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Ramanna KM, Sumanth TS, Konda D, Eerike M, Kalaiselvan SP, Elangovan C, Konda VGR, Janti SS. Effect of combining histaglobulin with antihistamines in patients with chronic spontaneous urticaria in a tertiary care hospital setting- a randomized trial. Arch Dermatol Res 2025; 317:516. [PMID: 40024926 DOI: 10.1007/s00403-025-04045-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/18/2024] [Revised: 02/07/2025] [Accepted: 02/12/2025] [Indexed: 03/04/2025]
Abstract
Chronic Spontaneous Urticaria (CSU) significantly affects quality of life. This study evaluates the efficacy of histaglobulin combined with antihistamine (bilastine) versus antihistamine alone in CSU management. A single-center, open-label, randomized trial was conducted in the dermatology OPD of a tertiary care hospital from September 2023 to August 2024. Fifty-seven eligible participants aged > 18 years with symptoms persisting > 6 weeks were randomized into test -histaglobulin plus bilastine group(n = 27) or bilastine alone (n = 30) groups. The test group received weekly histaglobulin injections and daily bilastine (20 mg), while the bilastine alone group received bilastine 20 mg only. Baseline data, including demographics, CSU characteristics, and Urticaria Activity Score (UAS), were recorded. Investigations included blood counts, renal/liver/thyroid function tests, serum IgE, absolute eosinophil count (AEC), vitamin B12 levels, and Autologous Serum Skin Test (ASST). UAS7 scores were recorded weekly for 6 weeks. The primary outcome was the change in UAS, with secondary outcomes assessing symptom control, and adverse events. A total 57 participants were recruited, with 27 and 30 in the test and bilastine alone group respectively. The combination of Histaglobulin with bilastine demonstrated significantly greater efficacy compared to bilastine alone. By Week 6, the Histaglobulin group achieved an 89.9% reduction in UAS versus 59.4% in the bilastine alone group (p < 0.01), with faster symptom resolution observed from Week 2. Laboratory findings showed notable reductions in serum IgE and AEC in the Histaglobulin group, suggesting enhanced modulation of allergic responses. No adverse effects were reported, highlighting the therapy's safety. The addition of Histaglobulin to bilastine provides a superior and well-tolerated treatment option for CSU, delivering faster symptom relief and better disease control. These findings support its use as an effective adjunctive therapy, warranting further investigation in larger sample size studies.
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Affiliation(s)
- Kalpana Mali Ramanna
- Department of Pharmacology, Neelima Institute of Medical Sciences, Hyderabad, Telangana, India
- Department of Pharmacology, All India Institute of Medical Sciences, Bibinagar, Hyderabad, Telangana, 508126, India
| | - Thota Sai Sumanth
- Department of Dermatology, All India Institute of Medical Sciences, Bibinagar, Hyderabad, Telangana, 508126, India
| | - Deepthi Konda
- Department of Dermatology, All India Institute of Medical Sciences, Bibinagar, Hyderabad, Telangana, 508126, India
| | - Madhavi Eerike
- Department of Pharmacology, All India Institute of Medical Sciences, Bibinagar, Hyderabad, Telangana, 508126, India.
| | | | - Chandru Elangovan
- Department of Pharmacology, Neelima Institute of Medical Sciences, Hyderabad, Telangana, India
- Department of General Surgery, S V Medical College, Tirupathi, Andhra Pradesh, India
| | - Venu Goapala Rao Konda
- Department of Pharmacology, Neelima Institute of Medical Sciences, Hyderabad, Telangana, India
| | - Siddharam S Janti
- Department of Ophthalmology, All India Institute of Medical Sciences, Bibinagar, Hyderabad, Telangana, 508126, India
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Weller K, Winders T, McCarthy J, Raftery T, Saraswat P, Constantinescu C, Balp MM, Bernstein JA. Urticaria Voices: Real-World Experience of Patients Living with Chronic Spontaneous Urticaria. Dermatol Ther (Heidelb) 2025:10.1007/s13555-025-01348-8. [PMID: 40019716 DOI: 10.1007/s13555-025-01348-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/18/2024] [Accepted: 01/23/2025] [Indexed: 03/01/2025] Open
Abstract
INTRODUCTION The impact of chronic spontaneous urticaria (CSU) on patients' health-related quality of life (HRQoL) is well documented. However, considerable gaps remain in understanding the experience, perception and needs of patients with CSU. In this study, we investigate the perspective of patients with CSU about the disease journey, treatment and management of the condition as well as the physical and psychosocial impact of the disease. METHODS A multinational, cross-sectional online survey was completed by patients with chronic urticaria (CU) and physicians treating CU. This analysis focuses on data from the patients with CSU. The patient survey included customized questions and a validated patient-reported outcomes measure, the Urticaria Control Test (UCT). RESULTS A total of 582 patients with CSU (62% women; mean [standard deviation, SD] age: 42.2 [11.9] years) completed the online survey. Patients reported a mean (SD) diagnostic delay of 2 (5.4) years and saw 6.1 (8.9) physicians. The majority (79%) of patients were on antihistamines, of which 84% were inadequately controlled (UCT score of < 12) and reported a significantly higher negative impact of CSU on the HRQoL domains than adequately controlled patients, with the highest impact on mental and emotional well-being and social life and intimate relationships. More than half (55%) of the patients experienced angioedema with a mean (SD) of 7.7 (14.0) episodes per year. In addition, sleeping problems (62%), pain (55%) and fatigue (49%) were frequently reported physical symptoms during an exacerbation. CONCLUSION Patients with CSU experience substantial burden due to delayed diagnosis, insufficient symptom control (despite treatment) as well as mental and emotional well-being and social impact, particularly when uncontrolled. Early diagnosis and patient-centered approaches to symptom management and disease control should be prioritized to minimize the negative impact of CSU on patients' life.
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Affiliation(s)
- Karsten Weller
- Institute of Allergology, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany
| | - Tonya Winders
- Allergy and Asthma Network, Fairfax, USA
- Global Allergy and Airways Patient Platform, Vienna, Austria
| | | | | | | | | | | | - Jonathan A Bernstein
- Division of Rheumatology, Allergy and Immunology, University of Cincinnati, Cincinnati, OH, USA.
- Bernstein Allergy Group and Clinical Research Centre, Cincinnati, OH, USA.
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Hide M, Uda A, Maki F, Miyakawa N, Kohli RK, Gupta S, Krupsky K, Balkaran B, Balp MM. Prevalence and Burden of Chronic Spontaneous Urticaria in Japan: A Cross-Sectional Study. J Clin Med 2025; 14:1162. [PMID: 40004692 PMCID: PMC11856825 DOI: 10.3390/jcm14041162] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/26/2024] [Revised: 01/10/2025] [Accepted: 02/08/2025] [Indexed: 02/27/2025] Open
Abstract
Background: Real-world data on the prevalence and burden of patients with chronic spontaneous urticaria (CSU) are limited in Japan. This study aimed to estimate CSU prevalence and assess its humanistic and economic burden. Methods: This analysis utilized data from Japanese adult respondents self-reporting physician-diagnosed CSU collected through the 2019 National Health and Wellness Survey. The weighted 12-month prevalence was estimated using 2018 international census projections. Outcomes included the SF-12v2 (physical and mental component summary [PCS and MCS] scores), health utility index (SF-6D and EQ-5D), Dermatology Life Quality Index (DLQI), Generalized Anxiety Disorder-7, Patient Health Questionnaire-9, Work Productivity and Activity Impairment scores at data collection, and healthcare resource utilization over the past 6 months. Results: Among 30,006 respondents, 334 reported having CSU, of whom 62.3% were female. The mean (SD) age at data collection and CSU diagnosis was 50.8 (15.3) and 39.2 (14.9) years, respectively. The weighted prevalence of CSU was 1.1%. The mean (SD) PCS and MCS scores were 50.3 (7.0) and 45.1 (10.0), respectively. The mean (SD) health utility measures (SF-6D and EQ-5D) were 0.71 (0.13) and 0.79 (0.18), respectively. The mean (SD) DLQI score was 3.8 (6.0). More than 40% of patients reported mild/moderate/severe anxiety and depression. The mean % (SD) scores for absenteeism, presenteeism, overall work impairment, and activity impairment were 7.6 (17.6), 27.2 (27.2), 30.3 (29.6), and 28.5 (27.8), respectively. Approximately 90.0% of patients visited healthcare providers, including emergency room visits (6.9%) and hospitalizations (9.9%). Conclusions: This study provides insights into the diagnosed prevalence and burden of CSU in Japan, highlighting its impact on patients' lives.
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Affiliation(s)
- Michihiro Hide
- Department of Dermatology, Hiroshima Citizens Hospital, Hiroshima 730-8518, Japan
| | - Akihito Uda
- Novartis Pharma K.K., Tokyo 105-6333, Japan; (A.U.); (F.M.); (N.M.)
| | - Fuyuko Maki
- Novartis Pharma K.K., Tokyo 105-6333, Japan; (A.U.); (F.M.); (N.M.)
| | - Noriko Miyakawa
- Novartis Pharma K.K., Tokyo 105-6333, Japan; (A.U.); (F.M.); (N.M.)
| | | | - Shaloo Gupta
- Oracle Life Sciences, Austin, TX 78741, USA; (S.G.); (K.K.); (B.B.)
| | - Kathryn Krupsky
- Oracle Life Sciences, Austin, TX 78741, USA; (S.G.); (K.K.); (B.B.)
| | - Bridget Balkaran
- Oracle Life Sciences, Austin, TX 78741, USA; (S.G.); (K.K.); (B.B.)
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Takahashi H, Fukunaga A, Hayama K, Sasajima T, Fujishige A, Ichishita R, Tomimatsu N, Hua E, Varanasi V, Burciu A, Hide M, Severin T. Long term safety and efficacy of ligelizumab in the treatment of Japanese patients with chronic spontaneous urticaria. Allergol Int 2025; 74:136-143. [PMID: 39327219 DOI: 10.1016/j.alit.2024.09.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/29/2024] [Revised: 07/25/2024] [Accepted: 08/10/2024] [Indexed: 09/28/2024] Open
Abstract
BACKGROUND In Japan, urticaria is a common skin disorder with chronic spontaneous urticaria (CSU) being the most frequent subtype. This study evaluated the safety of ligelizumab (anti-IgE monoclonal antibody) in Japanese CSU patients. METHODS This was a Phase 3 multicenter, open-label, single-arm 52-week study in adult Japanese patients with CSU inadequately controlled with locally approved doses of H1-antihistamines. The primary objective reported the safety of ligelizumab 120 mg subcutaneously every 4 weeks, by evaluation of treatment emergent adverse events (TEAE). Secondary objectives evaluated efficacy by absolute change from baseline (CFB) in weekly urticaria activity score (UAS7), and the proportion of patients with UAS7 = 0, and dermatology life quality index (DLQI) = 0-1 over time. RESULTS From a total of 66 CSU patients (80.3% females; mean ± SD age 46.4 ± 13.2 years; mean ± SD baseline UAS7 28.7 ± 6.5) enrolled, 53 patients (80.3%) reported ≥1 TEAE during the study, with no severe or serious adverse events, no anaphylaxis events and low frequency of TEAEs leading to treatment discontinuations (6.1%). Absolute mean CFB of UAS7 showed a rapid onset of response at Week 4 (-14.2) with further improvement until end of treatment at Week 52 (-22.9). The proportion of patients achieving UAS7 = 0 improved over time (14.5% at Week 4; 50.0% at Week 52). A sizable proportion of patients achieved DLQI 0-1 with the first dose of ligelizumab (38.5%), and improvements observed throughout the study until Week 52 (68.8%). CONCLUSIONS Treatment with ligelizumab 120 mg was well-tolerated with mild to moderate adverse events and was efficacious in Japanese patients.
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Affiliation(s)
| | - Atsushi Fukunaga
- Department of Dermatology, Division of Medicine for Function and Morphology of Sensory Organs, Faculty of Medicine, Osaka Medical and Pharmaceutical University, Osaka, Japan
| | - Koremasa Hayama
- Department of Dermatology, Nihon University Itabashi Hospital, Tokyo, Japan
| | | | | | | | | | - Eva Hua
- China Novartis Institutes for Biomedical Research Co., Ltd., Shanghai, China
| | | | | | - Michihiro Hide
- Department of Dermatology, Hiroshima City Hiroshima Citizens Hospital, Hiroshima, Japan; Department of Dermatology, Hiroshima University, Hiroshima, Japan.
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Alotaibi HM, Alghamdi AS, Almutairi RT, Altamimi RM, Osailan R. The prevelance of food allergy in patients with chronic idiopathic urticaria: a systematic review and meta-analysis. Arch Dermatol Res 2024; 317:132. [PMID: 39680174 DOI: 10.1007/s00403-024-03613-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/20/2024] [Revised: 10/20/2024] [Accepted: 11/26/2024] [Indexed: 12/17/2024]
Abstract
Chronic idiopathic urticaria (CIU) is difficult for patients and healthcare professionals to manage owing to its chronic and unpredictable nature. While the exact cause of CIU is unknown, it is suggested that food allergies may contribute to the development of symptoms. To determine the prevalence of food allergy in patients diagnosed with CIU. This systematic review is reported in accordance with PRISMA guidelines. We included all relevant articles published from inception to April 2023 in Scopus, Midline, and Web of Science. The initial search yielded 282 articles, of which only seven were included after applying the inclusion and exclusion criteria. The systematic review included a total of 1551 patients with urticaria. Approximately 25% of patients with food allergies had CIU. The prevalence of a family history of atopy was 37.9% (range, 24.3-53.8%, P = 0.133). There was a significant risk of angioedema in patients with urticaria (P = 0.039). The risk of CIU among patients with food allergies was estimated to be one in every four patients with urticaria, with a similar risk among the adult and pediatric populations. Patients with food allergies and a history of atopy were at higher risk of CIU. Angioedema is a common concomitant manifestation associated with common urticaria. This knowledge is important to identify patients at higher risk of urticaria and implement the necessary management to minimize potential complications and maintain a controlled disease.
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Affiliation(s)
- Hend M Alotaibi
- Dermatology Department, King Saud University, Riyadh, Saudi Arabia.
| | - Asail S Alghamdi
- Dermatology Department, King Fahad Hospital, Albaha, Saudi Arabia
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Štrajtenberger M, Lugović-Mihić L, Stipić-Marković A, Artuković M, Glavina A, Pravica NB, Hanžek M, Sušić T, Tešija Kuna A, Nađ Bungić L. Assesment of Salivary and Serum Levels of HBD2 in Patients with Chronic Angioedema. J Clin Med 2024; 13:7552. [PMID: 39768474 PMCID: PMC11728209 DOI: 10.3390/jcm13247552] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/31/2024] [Revised: 12/06/2024] [Accepted: 12/09/2024] [Indexed: 01/16/2025] Open
Abstract
Background/Objectives: Human β-defensin 2 (HBD2) is a protein that plays an important role in activating the immune system by modulating spinal pathways and the inflammatory response. According to previous research, HBD2 was proven to be important in chronic spontaneous urticaria (CSU) (their values were significantly elevated in CSU patients, with a significant correlation between HBD2 levels and the percentage of peripheral basophils, suggesting that elevated HBD2 levels may be a potential marker of basophil and mast cell activation), which led us to additional research on the HBD2 molecule in isolated chronic angioedema. The aim of this research is to examine HBD2 values in the saliva and serum of patients with isolated angioedema, as a potential biomarker of the disease. Methods: This cross-sectional study involved a total of 102 participants, involving three groups: 33 patients with isolated chronic non-hereditary angioedema (AE) (defined as sudden onset of localized edema without chronic urticaria), 33 patients with angioedema associated with chronic urticaria (CU+AE), and 35 healthy participants (controls, CTRL). They provided a saliva sample to determine HBD2 levels using an ELISA (Enzyme-Linked Immunosorbent Assay). Subsequently, a peripheral blood sample (serum) was taken from the participants to determine HBD2 levels using the same ELISA. Results: Salivary HBD2 levels were significantly higher in those with CU+AE than in the CTRL (p = 0.019). While salivary HBD2 values differed between those with angioedema and CTRL, the serum HBD2 values did not. Also, no correlation between the levels of HBD2 in saliva and serum was found. Conclusions: Since we found that salivary HBD2 values were significantly higher in those with CU+AE than in CTRL, this points to a possible role of the HBD2 molecule in pathogenesis of AE (namely, that it induces degranulation in mast cells and vascular permeability, and has antimicrobial properties) Therefore, more research is needed to determine how reliable salivary HBD2 measurement is, as well as its significance.
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Affiliation(s)
- Maja Štrajtenberger
- Department of Pulmology, Special Hospital for Pulmonary Diseases, 10000 Zagreb, Croatia; (M.Š.); (L.N.B.)
| | - Liborija Lugović-Mihić
- Department of Dermatovenereology, University Hospital Center Sestre Milosrdnice, 10000 Zagreb, Croatia
- School of Dental Medicine, University of Zagreb, 10000 Zagreb, Croatia
| | - Asja Stipić-Marković
- Department for Respiratory Infections, Dr. Fran Mihaljević University Hospital for Infections Diseases, 10000 Zagreb, Croatia;
| | - Marinko Artuković
- Faculty of Dental Medicine and Health Osijek, 31000 Osijek, Croatia;
| | - Ana Glavina
- Department of Dental Medicine, University Hospital of Split, 21000 Split, Croatia;
- Department of Oral Medicine, Study of Dental Medicine, School of Medicine, University of Split, 21000 Split, Croatia
| | - Nika Barbara Pravica
- Department of Emergency Medicine, University Hospital Center Sestre Milosrdnice, 10000 Zagreb, Croatia;
| | - Milena Hanžek
- Department of Clinical Chemistry, University Hospital Center Sestre Milosrdnice, 10000 Zagreb, Croatia; (M.H.); (T.S.); (A.T.K.)
| | - Tamara Sušić
- Department of Clinical Chemistry, University Hospital Center Sestre Milosrdnice, 10000 Zagreb, Croatia; (M.H.); (T.S.); (A.T.K.)
| | - Andrea Tešija Kuna
- Department of Clinical Chemistry, University Hospital Center Sestre Milosrdnice, 10000 Zagreb, Croatia; (M.H.); (T.S.); (A.T.K.)
| | - Lara Nađ Bungić
- Department of Pulmology, Special Hospital for Pulmonary Diseases, 10000 Zagreb, Croatia; (M.Š.); (L.N.B.)
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Mosnaim G, Patil D, Kuruvilla M, Hetherington J, Keal A, Mehlis S. Patient and physician perspectives on disease burden in chronic spontaneous urticaria: A real-world US survey. Ann Allergy Asthma Immunol 2024:S1081-1206(24)01722-8. [PMID: 39662669 DOI: 10.1016/j.anai.2024.11.028] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/21/2024] [Revised: 11/26/2024] [Accepted: 11/26/2024] [Indexed: 12/13/2024]
Abstract
BACKGROUND Chronic spontaneous urticaria (CSU) is frequently associated with severe disease-related symptoms that negatively affect quality of life, but patients and physicians may differ in their opinion on CSU burden. OBJECTIVE To describe the clinical and humanistic burden associated with CSU and level of agreement between patient and physician perceptions of disease burden and treatment satisfaction. METHODS This cross-sectional, survey-based study of US physicians and their adult patients with CSU included data collected in the Adelphi CSU Disease Specific Programme from 2020 to 2021. Overall, 1082 patient record forms completed by 110 physicians (including 40 allergists/immunologists, 50 dermatologists, and 20 primary care physicians) and 474 matched patient-reported questionnaires were included. Paired physician-patient records were used to determine agreement on disease burden and treatment satisfaction. RESULTS Patients with CSU often experienced physician-reported itching (66%) and hives (49%) and had a history of angioedema (23%). Although current CSU severity had largely improved since diagnosis, many patients and physicians continued to report moderate/severe current disease symptoms (46% and 30%, respectively). Moderate/severe disease had greater impacts on quality of life, sleep, work impairment, and treatment satisfaction than mild disease. Most patients and physicians agreed on symptom severity (61%-74%), with disagreement largely due to physicians underreporting severity relative to patients. Patient/physician agreement on treatment satisfaction was highest with mild CSU severity (82%), mild hive severity (80%), and omalizumab or other biologic treatment (87%). CONCLUSION Moderate/severe CSU was associated with greater disease burden and lower treatment satisfaction than mild CSU. Physicians more frequently underreported CSU severity compared with their patients.
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Affiliation(s)
| | - Dhaval Patil
- Novartis Pharmaceuticals Corp, East Hanover, New Jersey
| | | | | | - Aaron Keal
- Adelphi Real World, Bollington, United Kingdom
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Kolkhir P, Bonnekoh H, Metz M, Maurer M. Chronic Spontaneous Urticaria: A Review. JAMA 2024; 332:1464-1477. [PMID: 39325444 DOI: 10.1001/jama.2024.15568] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 09/27/2024]
Abstract
Importance Chronic spontaneous urticaria affects approximately 1% of the general population worldwide, including approximately 3 million people in the US, impairs patients' quality of life, and is associated with multiple comorbidities. Observations Chronic spontaneous urticaria affects patients of any age but is most common in females aged 30 to 50 years. Diagnosis is based on clinical presentation, ie, spontaneously recurring wheals, angioedema, or both. Chronic spontaneous urticaria persists for more than 1 year in most patients (1 or repeated episodes) and may present with comorbidities including chronic inducible urticaria (>10%), autoimmune thyroiditis (approximately 20%), metabolic syndrome (6%-20%), and anxiety (10%-31%) and depression (7%-29%). Known autoimmune endotypes (subtypes of urticaria defined by distinct pathogenesis) of chronic spontaneous urticaria are mediated by mast cell-activating IgE and/or IgG autoantibodies (>50%). Approximately 40% of patients with chronic spontaneous urticaria have a Dermatology Life Quality Index of more than 10, corresponding to a very large or extremely large negative effect on quality of life. Second-generation H1 antihistamines are first-line treatment; partial or complete response, defined as a reduction in urticaria symptoms of greater than 50%, is observed in approximately 40% of patients. The 2022 international urticaria guideline recommends the monoclonal anti-IgE antibody omalizumab as second-line treatment for antihistamine-refractory chronic spontaneous urticaria. However, at least 30% of patients have an insufficient response to omalizumab, especially those with IgG-mediated autoimmune urticaria. Cyclosporine, used off-label, can improve symptoms in approximately 54% to 73% of patients, especially those with autoimmune chronic spontaneous urticaria and nonresponse to omalizumab, but has adverse effects such as kidney dysfunction and hypertension. Conclusions and Relevance Chronic spontaneous urticaria is an inflammatory skin disease associated with medical and psychiatric comorbidities and impaired quality of life. Second-generation H1 antihistamines are first-line treatment, omalizumab is second-line treatment, and cyclosporine is third-line treatment for chronic spontaneous urticaria.
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Affiliation(s)
- Pavel Kolkhir
- Urticaria Center of Reference and Excellence (UCARE), Institute of Allergology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany
- Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Immunology and Allergology, Berlin, Germany
| | - Hanna Bonnekoh
- Urticaria Center of Reference and Excellence (UCARE), Institute of Allergology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany
- Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Immunology and Allergology, Berlin, Germany
| | - Martin Metz
- Urticaria Center of Reference and Excellence (UCARE), Institute of Allergology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany
- Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Immunology and Allergology, Berlin, Germany
| | - Marcus Maurer
- Urticaria Center of Reference and Excellence (UCARE), Institute of Allergology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany
- Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Immunology and Allergology, Berlin, Germany
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Chernyshov PV, Finlay AY, Tomas-Aragones L, Zuberbier T, Kocatürk E, Manolache L, Pustisek N, Svensson A, Marron SE, Sampogna F, Bewley A, Salavastru C, Koumaki D, Augustin M, Linder D, Abeni D, Salek SS, Szepietowski J, Jemec GB. Quality of life measurement in urticaria: Position statement of the European Academy of Dermatology and Venereology Task Forces on Quality of Life and Patient-Oriented Outcomes and Urticaria and Angioedema. J Eur Acad Dermatol Venereol 2024; 38:2056-2072. [PMID: 38855825 DOI: 10.1111/jdv.20157] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/27/2024] [Accepted: 05/03/2024] [Indexed: 06/11/2024]
Abstract
The European Academy of Dermatology and Venereology (EADV) Task Forces on quality of life (QoL) and patient-oriented outcomes and on urticaria and angioedema recommendations for the assessment of Health-related (HR) QoL in all patients with urticaria in research and practice are as follows: to use the DLQI for adults and the CDLQI for children as dermatology-specific and the CU-Q2oL as a disease-specific HRQoL instruments in urticaria; to use generic instruments to provide comparison of data on urticaria with non-dermatologic diseases, or to compare with healthy volunteers or the general population; to select validated HRQoL instruments with appropriate age limits; to present exact numeric data for HRQoL results; correct title of any HRQoL instrument should be used, along with its correct abbreviation and the reference to its original publication, where possible. The EADV TFs discourage the use of non-validated HRQoL instruments and modified HRQoL instruments that have not undergone standard validation.
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Affiliation(s)
- P V Chernyshov
- Department of Dermatology and Venereology, National Medical University, Kiev, Ukraine
| | - A Y Finlay
- Division of Infection and Immunity, School of Medicine, Cardiff University, Cardiff, UK
| | - L Tomas-Aragones
- Department of Psychology, University of Zaragoza, Zaragoza, Spain
| | - T Zuberbier
- Institute of Allergology, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität Zu Berlin, Berlin, Germany
- Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Allergology and Immunology, Berlin, Germany
| | - E Kocatürk
- Institute of Allergology, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität Zu Berlin, Berlin, Germany
- Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Allergology and Immunology, Berlin, Germany
- Department of Dermatology, Koç University School of Medicine, Istanbul, Turkey
| | - L Manolache
- Department of Dermatology, Dali Medical, Bucharest, Romania
| | - N Pustisek
- Children's Hospital Zagreb, Zagreb, Croatia
| | - A Svensson
- Department of Dermatology and Venereology, Skane University Hospital, Malmö, Sweden
| | - S E Marron
- Department of Dermatology, University Hospital Miguel Servet, Aragon Psychodermatology Research Group (GAI+PD), Zaragoza, Spain
| | - F Sampogna
- Clinical Epidemiology Unit, IDI-IRCCS, Rome, Italy
| | - A Bewley
- Barts Health NHS Trust, London, UK
- Queen Mary University, London, UK
| | - C Salavastru
- Department of Paediatric Dermatology, Colentina Clinical Hospital, "Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania
| | - D Koumaki
- Department of Dermatology and Venereology, University Hospital of Heraklion, Heraklion, Crete, Greece
| | - M Augustin
- Institute for Health Services Research in Dermatology and Nursing (IVDP), University Medical Center Hamburg-Eppendorf, Hamburg, Germany
| | - D Linder
- University Clinic for Medical Psychology and Psychotherapy, Medical University of Graz, Graz, Austria
| | - D Abeni
- Clinical Epidemiology Unit, IDI-IRCCS, Rome, Italy
| | - S S Salek
- School of Life & Medical Sciences, University of Hertfordshire, Hatfield, UK
| | - J Szepietowski
- Department of Dermatology, Wroclaw Medical University, Wroclaw, Poland
| | - G B Jemec
- Department of Dermatology, Zealand University Hospital, Roskilde, Denmark
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10
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Casale TB, Trzaskoma B, Holden M, Bernstein JA, Maurer M. Does angioedema in patients with chronic spontaneous urticaria impact response to omalizumab? World Allergy Organ J 2024; 17:100943. [PMID: 39193419 PMCID: PMC11347842 DOI: 10.1016/j.waojou.2024.100943] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/18/2024] [Revised: 06/20/2024] [Accepted: 07/20/2024] [Indexed: 08/29/2024] Open
Abstract
The presence of angioedema, or deep skin swelling, in addition to hives (wheals) in patients with chronic spontaneous urticaria (CSU) can complicate disease management. There is evidence that omalizumab is effective for patients with CSU with angioedema, but the time to a clinically meaningful response has not been assessed. This post hoc analysis examined data from the phase 3, randomized, double-blind ASTERIA I and ASTERIA II studies: patients with CSU with hives were grouped by presence (n = 216) or absence of angioedema (n = 265) at baseline. The time to minimally important difference (MID, change from baseline of ≥11 points) in weekly Urticaria Activity Score (UAS7) was analyzed using Kaplan-Meier analyses. Median time to MID for omalizumab 300 mg was similar in patients with and without angioedema. Median time to MID for omalizumab 150 mg was similar to 300 mg for patients without angioedema, and was longer for patients with angioedema. Therefore, the response to omalizumab for patients with CSU with angioedema was dose dependent. We recommend that the best approach for clinicians, in line with guidelines, would be initial administration of omalizumab 300 mg every 4 weeks for all patients. Clinical trials registration Clinicaltrials.gov NCT01287117 (registered 27 January 2011) and NCT01292473 (registered 7 February 2011).
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Affiliation(s)
- Thomas B. Casale
- Division of Allergy and Immunology, University of South Florida, Tampa, FL, USA
| | | | | | | | - Marcus Maurer
- Institute of Allergology, Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany
- Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Immunology and Allergology, Berlin, Germany
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11
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Pyatilova P, Hackler Y, Aulenbacher F, Asero R, Bauer A, Bizjak M, Day C, Dissemond J, Du-Thanh A, Fomina D, Giménez-Arnau AM, Grattan C, Gregoriou S, Hawro T, Kasperska-Zajac A, Khoshkhui M, Kocatürk E, Kovalkova E, Kulthanan K, Kuznetsova E, Makris M, Mukhina O, Pesqué D, Peter J, Salameh P, Siebenhaar F, Sikora A, Staubach P, Tuchinda P, Zamłyński M, Weller K, Maurer M, Kolkhir P. Non-Skin Related Symptoms Are Common in Chronic Spontaneous Urticaria and Linked to Active and Uncontrolled Disease: Results From the Chronic Urticaria Registry. THE JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY. IN PRACTICE 2024; 12:1890-1899.e3. [PMID: 38670260 DOI: 10.1016/j.jaip.2024.04.027] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 10/17/2023] [Revised: 04/15/2024] [Accepted: 04/16/2024] [Indexed: 04/28/2024]
Abstract
BACKGROUND Chronic spontaneous urticaria (CSU) can present with non-skin related symptoms (NSRS), including recurrent unexplained fever, joint, bone, or muscle pain (JBMP), and malaise, which also occur in other conditions that manifest with wheals (eg, urticarial vasculitis or autoinflammatory disorders) or without wheals (eg, infection). OBJECTIVE We sought to determine the rate of patients with CSU affected by fever, JBMP, and malaise, their trigger factors, links with clinical and laboratory characteristics, and their impact on everyday life and treatment responses. METHODS We analyzed baseline data from the Chronic Urticaria Registry of 2,521 patients with CSU who were aged 16 years or older. RESULTS One third of CSU patients (31.2%; 786 of 2,521) had one or more NSRS, including recurrent fever (5.3%), JBMP (19.1%), and/or malaise (18.6%). In a multivariable analysis, having one or more of these NSRS correlated with food and infection as trigger factors of urticaria (adjusted odds ratio [aOR] = 1.7 and 1.5), wheals of 24 hours or greater duration (aOR = 2.5), sleep disturbance (aOR = 2.4), anxiety (aOR = 2.8), comorbid atopic dermatitis (aOR = 2.1), gastrointestinal disease (aOR = 1.8), elevated leukocytes (aOR = 1.7) and erythrocyte sedimentation rate (aOR = 1.5). In a bivariate analysis, these NSRS were additionally associated with higher disease activity (weekly Urticaria Activity Score, median: 21 vs 14; P = .009), longer disease duration (years, median: 2 vs 1; P = .001), the presence of angioedema (74.6% vs 58.7%; P < .001), worse quality of life (Chronic Urticaria Quality of Life Questionnaire, median: 42 vs 29; P < .001) and more frequent poor control of CSU (78% vs 69%; P < .001). CONCLUSIONS The presence of NSRS in a subpopulation of patients with CSU points to the need for better control of the disease, exclusion of comorbid conditions, and/or exclusion of urticarial vasculitis and urticarial autoinflammatory diseases.
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Affiliation(s)
- Polina Pyatilova
- Institute of Allergology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany; Fraunhofer Institute for Translational Medicine and Pharmacology, Immunology and Allergology, Berlin, Germany
| | - Yana Hackler
- Institute of Allergology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany; Fraunhofer Institute for Translational Medicine and Pharmacology, Immunology and Allergology, Berlin, Germany
| | - Felix Aulenbacher
- Institute of Allergology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany; Fraunhofer Institute for Translational Medicine and Pharmacology, Immunology and Allergology, Berlin, Germany
| | - Riccardo Asero
- Ambulatorio di Allergologia, Clinica san Carlo, Paderno Dugnano, Italy
| | - Andrea Bauer
- Department of Dermatology, University Hospital Carl Gustav Carus, Technical University Dresden, Dresden, Germany
| | - Mojca Bizjak
- Division of Allergy, University Clinic of Respiratory and Allergic Diseases Golnik, Golnik, Slovenia
| | - Cascia Day
- Urticaria Center of Reference and Excellence, Division of Allergy and Clinical Immunology, Department of Medicine, University of Cape Town, Cape Town, South Africa; Urticaria Center of Reference and Excellence, Allergy and Immunology Unit, University of Cape Town, Lung Institute, Cape Town, South Africa
| | - Joachim Dissemond
- Department of Dermatology, Venerology, and Allergology, University of Essen, Essen, Germany
| | - Aurélie Du-Thanh
- University of Montpellier and University Hospital of Montpellier, Dermatology Department, Montpellier, France
| | - Daria Fomina
- Moscow City Research and Practical Center of Allergology and Immunology, Moscow Healthcare Department, City Clinical Hospital 52, Moscow, Russia; Department of Clinical Immunology and Allergology, I.M., Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia; Department of Pulmonology, Astana Medical University, Astana, Kazakhstan
| | - Ana M Giménez-Arnau
- Department of Dermatology, Hospital del Mar and Research Institute, Barcelona, Universitat Pompeu Fabra, Barcelona, Spain
| | - Clive Grattan
- St John's Institute of Dermatology, Guy's Hospital, London, United Kingdom
| | - Stamatis Gregoriou
- Faculty of Medicine, National and Kapodistrian University of Athens, Andreas Sygros Hospital, Athens, Greece
| | - Tomasz Hawro
- Department of Dermatology, Allergology, and Venereology, Institute and Comprehensive Center for Inflammation Medicine, University Medical Center Schleswig-Holstein, Lübeck, Germany
| | - Alicja Kasperska-Zajac
- European Center for Diagnosis and Treatment of Urticaria/Angioedema (GA(2)LEN Urticaria Centers of Reference and Excellence Network), Medical University of Silesia in Katowice, Katowice, Poland
| | - Maryam Khoshkhui
- Allergy Research Center, Mashhad University of Medical Science, Mashhad, Iran
| | - Emek Kocatürk
- Institute of Allergology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany; Fraunhofer Institute for Translational Medicine and Pharmacology, Immunology and Allergology, Berlin, Germany; Department of Dermatology, Koç University School of Medicine, Istanbul, Turkey
| | - Elena Kovalkova
- Moscow City Research and Practical Center of Allergology and Immunology, Moscow Healthcare Department, City Clinical Hospital 52, Moscow, Russia
| | - Kanokvalai Kulthanan
- Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
| | - Elizaveta Kuznetsova
- Moscow City Research and Practical Center of Allergology and Immunology, Moscow Healthcare Department, City Clinical Hospital 52, Moscow, Russia
| | - Michael Makris
- Allergy Unit, Second Department of Dermatology and Venereology, National and Kapodistrian University of Athens, University General Hospital "Attikon," Athens, Greece
| | - Olga Mukhina
- Moscow City Research and Practical Center of Allergology and Immunology, Moscow Healthcare Department, City Clinical Hospital 52, Moscow, Russia
| | - David Pesqué
- Department of Dermatology, Hospital del Mar Research Institute, Universitat Autònoma de Barcelona, Barcelona, Spain
| | - Jonny Peter
- Urticaria Center of Reference and Excellence, Division of Allergy and Clinical Immunology, Department of Medicine, University of Cape Town, Cape Town, South Africa; Urticaria Center of Reference and Excellence, Allergy and Immunology Unit, University of Cape Town, Lung Institute, Cape Town, South Africa
| | - Pascale Salameh
- Institute of Allergology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany; Fraunhofer Institute for Translational Medicine and Pharmacology, Immunology and Allergology, Berlin, Germany; Research Department, School of Medicine, Lebanese American University, Byblos, Lebanon; Institut National de Santé Publique d'Épidémiologie Clinique et de Toxicologie-Liban, Beirut, Lebanon; Department of Primary Care and Population Health, University of Nicosia Medical School, Nicosia, Cyprus; Faculty of Pharmacy, Lebanese University, Hadat, Lebanon
| | - Frank Siebenhaar
- Institute of Allergology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany; Fraunhofer Institute for Translational Medicine and Pharmacology, Immunology and Allergology, Berlin, Germany
| | - Agnieszka Sikora
- European Center for Diagnosis and Treatment of Urticaria/Angioedema (GA(2)LEN Urticaria Centers of Reference and Excellence Network), Medical University of Silesia in Katowice, Katowice, Poland
| | - Petra Staubach
- Department of Dermatology, Johannes Gutenberg University, Mainz, Germany
| | - Papapit Tuchinda
- Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
| | - Mateusz Zamłyński
- European Center for Diagnosis and Treatment of Urticaria/Angioedema (GA(2)LEN Urticaria Centers of Reference and Excellence Network), Medical University of Silesia in Katowice, Katowice, Poland
| | - Karsten Weller
- Institute of Allergology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany; Fraunhofer Institute for Translational Medicine and Pharmacology, Immunology and Allergology, Berlin, Germany
| | - Marcus Maurer
- Institute of Allergology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany; Fraunhofer Institute for Translational Medicine and Pharmacology, Immunology and Allergology, Berlin, Germany
| | - Pavel Kolkhir
- Institute of Allergology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany; Fraunhofer Institute for Translational Medicine and Pharmacology, Immunology and Allergology, Berlin, Germany.
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12
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Fukunaga A, Kishi Y, Arima K, Fujita H. Disease Control and Treatment Satisfaction in Patients with Chronic Spontaneous Urticaria in Japan. J Clin Med 2024; 13:2967. [PMID: 38792508 PMCID: PMC11121873 DOI: 10.3390/jcm13102967] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/26/2024] [Revised: 05/15/2024] [Accepted: 05/16/2024] [Indexed: 05/26/2024] Open
Abstract
Background: Chronic spontaneous urticaria (CSU), characterized by the recurrence of pruritic hives and/or angioedema for >6 weeks with no identifiable trigger, has a negative impact on health-related quality of life (HRQoL). Methods: The objective of this web-based cross-sectional study was to evaluate disease control, disease burden, and treatment satisfaction in Japanese adults with CSU using the Urticaria Control Test (UCT), HRQoL outcomes, and the Treatment Satisfaction Questionnaire for Medication-9 items (TSQM-9). Results: In total, 529 adults were included in the analysis (59.9% female), with a mean ± standard deviation (SD) in CSU duration of 13.2 ± 13.0 years. Based on UCT scores, two-thirds of patients had poor (score of 0-7; 23.6%) or insufficient (score of 8-11; 43.3%) symptom control, and one-third had good control (score of 12-16; 33.1%). Overall treatment satisfaction was not high, with mean ± SD TSQM-9 scores of 55.5 ± 17.6% for effectiveness, 68.2 ± 18.8% for convenience, and 59.2 ± 18.4% for global satisfaction. No apparent differences in TSQM-9 scores were observed between patients receiving different medications. HRQoL outcomes were worse among patients with poor/insufficient symptom control. Conclusions: Japanese adults with CSU have a high disease burden, and better treatment options are needed to increase treatment satisfaction.
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Affiliation(s)
- Atsushi Fukunaga
- Department of Dermatology, Division of Medicine for Function and Morphology of Sensory Organs, Faculty of Medicine, Osaka Medical and Pharmaceutical University, Osaka 569-8686, Japan;
| | - Yuko Kishi
- Specialty Care Medical, Sanofi K.K., Tokyo Opera City Tower, 3-20-2, Nishi Shinjuku, Tokyo 163-1488, Japan; (K.A.); (H.F.)
| | - Kazuhiko Arima
- Specialty Care Medical, Sanofi K.K., Tokyo Opera City Tower, 3-20-2, Nishi Shinjuku, Tokyo 163-1488, Japan; (K.A.); (H.F.)
| | - Hiroyuki Fujita
- Specialty Care Medical, Sanofi K.K., Tokyo Opera City Tower, 3-20-2, Nishi Shinjuku, Tokyo 163-1488, Japan; (K.A.); (H.F.)
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13
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Vincent D, Parsopoulou F, Martin L, Gaboriaud C, Demongeot J, Loules G, Fischer S, Cichon S, Germenis AE, Ghannam A, Drouet C. Hereditary angioedema with normal C1 inhibitor associated with carboxypeptidase N deficiency. THE JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY. GLOBAL 2024; 3:100223. [PMID: 38445235 PMCID: PMC10912455 DOI: 10.1016/j.jacig.2024.100223] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 08/07/2023] [Revised: 11/18/2023] [Accepted: 11/19/2023] [Indexed: 03/07/2024]
Abstract
Background Hereditary angioedema (HAE) is a potentially life-threatening disorder characterized by recurrent episodes of subcutaneous or submucosal swelling. HAE with normal C1 inhibitor (HAE-nC1-INH) is an underdiagnosed condition. Although the association with genetic variants has been identified for some families, the genetic causes in many patients with HAE-nC1-INH remain unknown. The role of genes associated with bradykinin catabolism is not fully understood. Objective We sought to investigate the biological parameters and the genes related to kallikrein-kinin system in families with a clinical phenotype of HAE-nC1-INH and presenting with a carboxypeptidase N (CPN) deficiency. Methods This study includes 4 families presenting with HAE-nC1-INH and CPN deficiency. Patients' clinical records were examined, biological parameters of kallikrein-kinin system were measured, and genetics was analyzed by next-generation sequencing and Sanger sequencing. Predictive algorithms (Human Splicing Finder, Sorting Intolerant From Tolerant, Polymorphism Phenotyping v2, MutationTaster, and ClinPred) were used to classify variants as affecting splicing, as benign to deleterious, or as disease-causing. Results Patients presented with angioedema and urticaria, mainly on face/lips, but also with abdominal pain or laryngeal symptoms. Affected patients displayed low CPN activity-30% to 50% of median value in plasma. We identified 3 variants of the CPN1 gene encoding the catalytic 55-kDa subunit of CPN: c.533G>A, c.582A>G, and c.734C>T. CPN deficiency associated with genetic variants segregated with HAE-nC1-INH symptoms in affected family members. Conclusions CPN1 gene variants are associated with CPN deficiency and HAE-nC1-INH symptoms in 4 unrelated families. Genetic CPN deficiency may contribute to bradykinin and anaphylatoxin accumulation, with synergistic effects in angioedema and urticarial symptoms.
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Affiliation(s)
- Denis Vincent
- Allergy and Internal Medicine Unit, University Hospital, Nîmes, France
- Centre de compétence, Centre de Référence des Angioedèmes (CREAK), Nîmes
| | | | - Ludovic Martin
- Dermatology Department, University Hospital, Angers, France
- Centre de Référence des Maladies Rares de la peau et des muqueuses d’origine génétique-Nord (MAGEC), filière FIMARAD, CHU Angers, Angers, France
| | | | | | | | - Sascha Fischer
- Human Genomics Research Group, Department of Biomedicine, University of Basel, Basel, Switzerland
- Institute of Medical Genetics and Pathology, University Hospital Basel, Basel, Switzerland
| | - Sven Cichon
- Human Genomics Research Group, Department of Biomedicine, University of Basel, Basel, Switzerland
- Institute of Medical Genetics and Pathology, University Hospital Basel, Basel, Switzerland
| | - Anastasios E. Germenis
- Department of Immunology and Histocompatibility, University of Thessaly, Larissa, Greece
| | | | - Christian Drouet
- Université Grenoble Alpes, CHU Grenoble Alpes, Grenoble, France
- Institut Cochin, INSERM, CNRS, Université Paris Cité, 75679, Paris, France
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14
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Baudy A, Raison-Peyron N, Serrand C, Crépy MN, Du-Thanh A. Impact of Chronic Spontaneous or Inducible Urticaria on Occupational Activity. Acta Derm Venereol 2024; 104:adv36122. [PMID: 38551378 PMCID: PMC11000653 DOI: 10.2340/actadv.v104.36122] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/05/2024] [Accepted: 02/27/2024] [Indexed: 04/02/2024] Open
Abstract
The impact of chronic urticaria on work has been scarcely reported, whereas its peak incidence is between the ages of 20 and 40. The aim of this study was to assess the occupational impact of chronic urticaria and its treatment, by combining objective and patient-reported data. A monocentric observational study was performed using questionnaires over a 1-year period from 2021 to 2022 in chronic urticaria patients who were in a period of professional activity and agreed to participate. Of the 88 patients included, 55.7% assessed the occupational impact of their chronic urticaria as significant, and even more severe when chronic urticaria was poorly controlled. Some 86% of patients had symptoms at work, in a third of cases aggravated by work. However, occupational physical factors were not associated with an aggravation of inducible chronic urticaria. A total of 20% reported treatment-related adverse effects affecting their work. Despite low absenteeism, presenteeism and reduced productivity were important (> 20%). Six patients (6.8%) had difficulties keeping their work. For 72.7% of the patients, the occupational physician was not informed. The occupational impact of chronic urticaria should be discussed during consultations, particularly when it is insufficiently controlled. The occupational physician should be informed in order to support patients' professional project.
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Affiliation(s)
- Amandine Baudy
- Department of Dermatology, University Hospital of Montpellier, Montpellier, France; University of Montpellier, Montpellier, France
| | - Nadia Raison-Peyron
- Department of Dermatology, University Hospital of Montpellier, Montpellier, France
| | - Chris Serrand
- University of Montpellier, Montpellier, France; Department of Biostatistics, Epidemiology, Public Health and Innovation in Methodology, Nîmes university hospital, Nîmes, France
| | - Marie-Noëlle Crépy
- Department of Dermatology, University Hospital of Centre of Paris, Cochin Hospital, AP-HP, Paris, France; Department of Occupational and Environmental Diseases, University Hospital of Centre of Paris, Hotel-Dieu Hospital, AP-HP, Paris, France
| | - Aurélie Du-Thanh
- Department of Dermatology, University Hospital of Montpellier, Montpellier, France; University of Montpellier, Montpellier, France.
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15
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Demir S, Eyice‐Karabacak D, Kocatürk E, Ünal D, Toprak İD, Korkmaz P, Aslan AF, İmren IG, Dikicier B, Kahveci N, Öztop N, Kara RÖ, İşsever H, Maurer M, Weller K, Gelincik A. Monitoring recurrent angioedema: Findings from the Turkish angioedema control test validation study. Clin Transl Allergy 2024; 14:e12342. [PMID: 38415974 PMCID: PMC10900914 DOI: 10.1002/clt2.12342] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/15/2023] [Revised: 01/29/2024] [Accepted: 01/31/2024] [Indexed: 02/29/2024] Open
Abstract
BACKGROUND Determination of control level in recurrent angioedema (RAE) is necessary to guide management. Here, we validated a Turkish version of the angioedema control test (AECT) for 4-week (AECT-4wk) and for 3-month (AECT-3mth) and assessed their utility in monitoring RAE. METHOD The recommended structured translation process for patient-reported outcome measures was completed. The final versions were administered to 51 patients with mast cell-mediated angioedema (MMAE) and 38 patients with hereditary angioedema, and the minimal clinically important difference (MCID) was determined. Additionally, anchor surveys comprising angioedema activity score for 28 days (AAS-28 day), visual analog score for angioedema control, Likert scale for the control level from the patient's perspective (LS-AEC), angioedema quality of life, short form-12 (SF-12) and patients' assessment of treatment sufficiency were applied. RESULTS The Turkish AECT versions showed good convergent validity with a substantial correlation with anchor tools and known-group validity. Excellent internal consistency and reproducibility were observed. Equal or more than 10 of 16 points scored with the AECT-4wk and AECT-3mth identified patients with well-controlled disease. The disease activity, control and burden parameters were consistent with the disease control level defined depending on the cut-off point 10 of AECT. Three-point changes in AECT-4wk and -3 mt could detect MCID in disease control in all patients. CONCLUSIONS Turkish AECT versions are valid and reliable tools for assessing and monitoring disease control in patients with RAE. The use of the Turkish versions of the AECT in routine patient care, clinical trials and angioedema research is recommended.
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Affiliation(s)
- Semra Demir
- Division of Immunology and Allergy DiseasesDepartment of Internal MedicineIstanbul Faculty of MedicineIstanbul UniversityIstanbulTurkey
| | - Deniz Eyice‐Karabacak
- Division of Immunology and Allergy DiseasesDepartment of Internal MedicineIstanbul Faculty of MedicineIstanbul UniversityIstanbulTurkey
| | - Emek Kocatürk
- Department of DermatologyKoç University Faculty of MedicineIstanbulTurkey
- Institute of AllergologyCharite‐UniversitatsmedizinCorporate Member of Freie Universitat Berlin and Humboldt‐Universitat zu BerlinBerlinGermany
- Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Immunology and AllergologyBerlinGermany
| | - Derya Ünal
- Division of Immunology and Allergy DiseasesDepartment of Internal MedicineIstanbul Faculty of MedicineIstanbul UniversityIstanbulTurkey
| | - İlkim Deniz Toprak
- Division of Immunology and Allergy DiseasesDepartment of Internal MedicineIstanbul Faculty of MedicineIstanbul UniversityIstanbulTurkey
| | - Pelin Korkmaz
- Division of Immunology and Allergy DiseasesDepartment of Internal MedicineIstanbul Faculty of MedicineIstanbul UniversityIstanbulTurkey
| | - Ayşe Feyza Aslan
- Division of Immunology and Allergy DiseasesDepartment of Internal MedicineIstanbul Faculty of MedicineIstanbul UniversityIstanbulTurkey
| | - Işıl Göğem İmren
- Division of Immunology and Allergy DiseasesDepartment of Internal MedicineIstanbul Faculty of MedicineIstanbul UniversityIstanbulTurkey
| | - Bahar Dikicier
- Faculty of MedicineDepartment of DermatologySakarya UniversitySakaryaTurkey
| | - Nevzat Kahveci
- Division of Immunology and Allergy DiseasesDepartment of Internal MedicineIstanbul Faculty of MedicineIstanbul UniversityIstanbulTurkey
| | - Nida Öztop
- Adult Allergy and Immunology ClinicBaşakşehir Pine and Sakura City HospitalIstanbulTurkey
| | - Rabia Öztaş Kara
- Faculty of MedicineDepartment of DermatologySakarya UniversitySakaryaTurkey
| | - Halim İşsever
- Istanbul Faculty of MedicineDepartment of Public HealthIstanbul UniversityIstanbulTurkey
| | - Marcus Maurer
- Institute of AllergologyCharite‐UniversitatsmedizinCorporate Member of Freie Universitat Berlin and Humboldt‐Universitat zu BerlinBerlinGermany
- Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Immunology and AllergologyBerlinGermany
| | - Karsten Weller
- Institute of AllergologyCharite‐UniversitatsmedizinCorporate Member of Freie Universitat Berlin and Humboldt‐Universitat zu BerlinBerlinGermany
| | - Aslı Gelincik
- Division of Immunology and Allergy DiseasesDepartment of Internal MedicineIstanbul Faculty of MedicineIstanbul UniversityIstanbulTurkey
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16
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Sánchez J, Jaimes F, García E, Zakzuk J, Cardona R, Velasquez M. Risk calculator of the clinical response to antihistamines in chronic urticaria: Development and internal validation. PLoS One 2024; 19:e0295791. [PMID: 38394074 PMCID: PMC10889609 DOI: 10.1371/journal.pone.0295791] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/17/2023] [Accepted: 11/28/2023] [Indexed: 02/25/2024] Open
Abstract
Early detection of CSU patients with low probability of a clinical response with antihistamines could undergo prompt initiation of therapeutic alternatives. The aim of the study was to develop and internally validate a model for predicting the clinical response to antihistamines in adult patients with chronic spontaneous urticaria (CSU), who consult allergology and dermatology care centers. A cohort of CSU patients, recruited from four participating centers, were followed up for 12 months. Fifteen candidate variables were selected to be included in the multivariate model and then internal validation was done with bootstrap analysis with 1000 simulations. The outcome variable, clinical response to antihistamines, was evaluated with the UAS (Urticaria Activity Score) scale for seven days: "No response to antihistamines" was defined as UAS7 ≥7 points after at least one month with a maximum dose of antihistamines, while "Response to antiH1" was defined as UAS7 ≤6 points for at least three months with the use of antiH1. A total of 790 patients were included. Among the different models analyzed, the model that included age, angioedema, anxiety/depression, time with the disease, NSAIDs (Non-steroidal anti-inflammatory drugs) intolerance, and UAS7 baseline was considered the one with the best performance (accuracy 0.675, HL 0.87, AUC 0.727). The internal validation analyses demonstrated good consistency of the model. In conclusion, this prediction model identifies the probability of response to antihistamines in patients with chronic spontaneous urticaria. The model could be useful for a personalized therapeutic approach according to individual patient risk.
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Affiliation(s)
- Jorge Sánchez
- Group of Clinical and Experimental Allergy, "IPS University" Clinic, University of Antioquia, Medellín, Colombia
| | - Fabian Jaimes
- Internal Medicine Department, “San Vicente” Clinic, University of Antioquia, Medellín, Colombia
| | | | - Josefina Zakzuk
- Health Economics Research Group, ALZAK Foundation, Immunological Research Institute, University of Cartagena, Cartagena, Colombia
| | - Ricardo Cardona
- Group of Clinical and Experimental Allergy, "IPS University" Clinic, University of Antioquia, Medellín, Colombia
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17
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Li PH, Au EYL, Cheong SL, Chung L, Fan KI, Ho MHK, Leung ASY, Chung MMH, Wong JCY, Coelho R. Hong Kong-Macau Severe Hives and Angioedema Referral Pathway. FRONTIERS IN ALLERGY 2023; 4:1290021. [PMID: 38125294 PMCID: PMC10731021 DOI: 10.3389/falgy.2023.1290021] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/06/2023] [Accepted: 11/20/2023] [Indexed: 12/23/2023] Open
Abstract
Background Urticaria (defined as the presence of hives, angioedema, or both) can be caused by a variety of etiologies ranging from more common conditions such as chronic spontaneous urticaria (CSU) to rarer conditions such as hereditary angioedema (HAE). Specialist referral may be necessary in cases of severe urticaria or HAE, but access to specialist services remains limited in certain regions, such as the Greater Bay Area (GBA) of China. To address this, the Hong Kong-Macau Severe Hives and Angioedema Referral Pathway (SHARP) was initiated by the Hong Kong Institute of Allergy and Macau Society of Dermatology to promote multidisciplinary collaboration and regional exchange of expertise in the diagnosis and management of severe urticaria. Methods A nominated task force of dermatologists and immunologists who manage patients with severe urticaria formulated the consensus statements (CS) using the Delphi method. The consensus was defined a priori as an agreement of ≥80%. Results A total of 24 CS were formulated, including four statements on classifications and definitions, seven statements on diagnosis, and 13 statements on management and referral. The definitions for acute/chronic urticaria and severe CSU were stated. Unnecessary investigations and inappropriate medications were discouraged. The characteristics and recommended approach to suspected bradykinergic angioedema were specified. Stepwise treatment options using second-generation antihistamines, omalizumab, or cyclosporin for patients with CSU were addressed, and the importance of access to HAE-specific medications was emphasized. Furthermore, an integrated referral pathway for patients with severe hives and angioedema was constructed. Conclusion The SHARP provides guidance for the management and specialist referral of patients with severe hives and angioedema in Hong Kong and Macau.
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Affiliation(s)
- Philip H. Li
- Division of Rheumatology and Clinical Immunology, Department of Medicine, The University of Hong Kong, Hong Kong, Hong Kong SAR, China
| | - Elaine Y. L. Au
- Division of Clinical Immunology, Department of Pathology, Queen Mary Hospital, Hong Kong, Hong Kong SAR, China
| | - Si-Leong Cheong
- Department of Dermatology, Centro Hospitalar Conde de São Januário, Macau, Macao SAR, China
| | - Ling Chung
- Department of Dermatology, Centro Hospitalar Conde de São Januário, Macau, Macao SAR, China
| | - Ka I. Fan
- Department of Dermatology, Centro Hospitalar Conde de São Januário, Macau, Macao SAR, China
| | - Marco H. K. Ho
- Lee Tak Hong Allergy Centre, Hong Kong Sanatorium & Hospital, Hong Kong, Hong Kong SAR, China
| | - Agnes S. Y. Leung
- Department of Paediatrics, Faculty of Medicine, The Chinese University of Hong Kong, Hong Kong, Hong Kong SAR, China
- Hong Kong Hub of Paediatric Excellence, The Chinese University of Hong Kong, Hong Kong, Hong Kong SAR, China
| | - Martin M. H. Chung
- Division of Dermatology, Department of Medicine, Queen Mary Hospital, Hong Kong, Hong Kong SAR, China
| | - Jane C. Y. Wong
- Division of Rheumatology and Clinical Immunology, Department of Medicine, The University of Hong Kong, Hong Kong, Hong Kong SAR, China
| | - Ricardo Coelho
- Department of Dermatology, Centro Hospitalar Conde de São Januário, Macau, Macao SAR, China
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18
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Casale TB, Gimenez-Arnau AM, Bernstein JA, Holden M, Zuberbier T, Maurer M. Omalizumab for Patients with Chronic Spontaneous Urticaria: A Narrative Review of Current Status. Dermatol Ther (Heidelb) 2023; 13:2573-2588. [PMID: 37776480 PMCID: PMC10613187 DOI: 10.1007/s13555-023-01040-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/26/2023] [Accepted: 09/13/2023] [Indexed: 10/02/2023] Open
Abstract
Chronic spontaneous urticaria (CSU) is a debilitating inflammatory disorder of the skin, characterized by a fluctuating natural history, a complex mechanism of action, and a significant burden on patients, including effect on quality of life, development of psychosocial disorders, and a range of comorbidities. Recent international guidelines recommend a therapeutic approach of first-line treatment with second generation H1-antihistamines and second-line treatment with the biologic omalizumab. Here, the salient aspects of CSU and current status of data for omalizumab for patients with CSU are reviewed, with a focus on mechanism of action, efficacy and real-world effectiveness (including patient outcomes, response, relapse, and remission), and safety (including consideration of the risk of anaphylaxis). The review also considers recent data on COVID-19, CSU, and omalizumab and presents our perspective on future needs. Overall, the data suggest that omalizumab is an effective and well-tolerated treatment for patients with CSU that provides benefits for a wide range of patients.
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Affiliation(s)
- Thomas B Casale
- Internal Medicine, Division of Allergy and Immunology, Morsani College of Medicine, University of South Florida, Tampa, FL, 33620, USA.
| | | | - Jonathan A Bernstein
- Department of Medicine, Division of Immunology Allergy Section, University of Cincinnati, Cincinnati, OH, USA
| | | | - Torsten Zuberbier
- Institute of Allergology, Charité - Universitätsmedizin Berlin, Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany
- Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Allergology and Immunology, Berlin, Germany
| | - Marcus Maurer
- Institute of Allergology, Charité - Universitätsmedizin Berlin, Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany
- Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Allergology and Immunology, Berlin, Germany
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19
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Buttgereit T, Vera C, Aulenbacher F, Church MK, Hawro T, Asero R, Bauer A, Bizjak M, Bouillet L, Dissemond J, Fomina D, Giménez-Arnau AM, Grattan C, Gregoriou S, Kulthanan K, Kasperska-Zajac A, Kocatürk E, Makris M, Kolkhir P, Weller K, Magerl M, Maurer M. Patients With Chronic Spontaneous Urticaria Who Have Wheals, Angioedema, or Both, Differ Demographically, Clinically, and in Response to Treatment-Results From CURE. THE JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY. IN PRACTICE 2023; 11:3515-3525.e4. [PMID: 37604426 DOI: 10.1016/j.jaip.2023.08.020] [Citation(s) in RCA: 6] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 04/06/2023] [Revised: 07/19/2023] [Accepted: 08/05/2023] [Indexed: 08/23/2023]
Abstract
BACKGROUND Patients with chronic spontaneous urticaria (CSU) have spontaneous wheals (W), angioedema (AE), or both, for longer than 6 weeks. Clinical differences between patients with standalone W, standalone AE, and W and AE (W+AE) remain incompletely understood. OBJECTIVE To compare W, AE, and W+AE CSU patients regarding demographics, disease characteristics, comorbidities, disease burden, and treatment response. METHODS Baseline data from 3,698 CSU patients in the ongoing, prospective, international, multicenter, observational Chronic Urticaria REgistry (CURE) were analyzed (data cut: September 2022). RESULTS Across all CSU patients, 59%, 36%, and 5% had W+AE, W, and AE, respectively. The W+AE patients, compared with W and AE patients, showed the lowest male-to-female ratio (0.33), higher rates of concomitant psychiatric disease (17% vs 11% vs 6%, respectively), autoimmune disease (13% vs 7% vs 9%, respectively), and nonsteroidal anti-inflammatory drug (NSAID) hypersensitivity (9% vs 5% vs 2%, respectively) and the highest disease impact. The W patients, compared with W+AE and AE patients, showed the lowest rates of concomitant hypertension (15% vs 21% vs 40%, respectively) and obesity (11% vs 16% vs 17%, respectively), the highest rate of concomitant inducible urticaria (24% vs 22% vs 6%, respectively), and shorter W duration. The AE patients, compared with W+AE and W patients, were older at disease onset, showed longer AE duration, and the best response to increased doses of H1-antihistamines (58% vs 24% vs 31%, respectively) and omalizumab (92% vs 67% vs 60%, respectively). CONCLUSIONS Our findings provide a better understanding of CSU phenotypes and may guide patient care and research efforts that aim to link them to pathogenic drivers.
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Affiliation(s)
- Thomas Buttgereit
- Institute of Allergology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany; Institute of Allergology, Fraunhofer Institute for Translational Medicine and Pharmacology (ITMP), Allergology and Immunology, Berlin, Germany
| | - Carolina Vera
- Institute of Allergology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany; Institute of Allergology, Fraunhofer Institute for Translational Medicine and Pharmacology (ITMP), Allergology and Immunology, Berlin, Germany
| | - Felix Aulenbacher
- Institute of Allergology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany; Institute of Allergology, Fraunhofer Institute for Translational Medicine and Pharmacology (ITMP), Allergology and Immunology, Berlin, Germany
| | - Martin K Church
- Institute of Allergology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany; Institute of Allergology, Fraunhofer Institute for Translational Medicine and Pharmacology (ITMP), Allergology and Immunology, Berlin, Germany
| | - Tomasz Hawro
- Institute and Comprehensive Center for Inflammation Medicine, Department of Dermatology, Allergology and Venerology, University Medical Center Schleswig-Holstein, Lübeck, Germany
| | - Riccardo Asero
- Department of Allergology, Clinica San Carlo, Paderno Dugnano, Milan, Italy
| | - Andrea Bauer
- Department of Dermatology, University Hospital, Carl Gustav Carus, Technical University Dresden, Dresden, Germany
| | - Mojca Bizjak
- Division of Allergy, University Clinic of Respiratory and Allergic Diseases Golnik, Golnik, Slovenia
| | - Laurence Bouillet
- CREAK (Centre national de référence des angioedèmes), Internal Medicine, CHU Grenobles Alpes, Grenoble, France
| | - Joachim Dissemond
- Department of Dermatology, Venerology and Allergology, University of Essen, Essen, Germany
| | - Daria Fomina
- Center of Allergy and Immunology, Clinical State Hospital 52, Moscow Healthcare Ministry, Moscow, Russian Federation; I. M. Sechenov First Moscow State Medical University (Sechenov University), Department of Clinical Immunology and Allergology, Moscow, Russian Federation
| | - Ana M Giménez-Arnau
- Department of Dermatology, Institut Mar de Investigacions Mèdiques, Universitat Pompeu Fabra, Barcelona, Spain
| | - Clive Grattan
- Guy's Hospital, St. John's Institute of Dermatology, London, UK
| | - Stamatios Gregoriou
- National and Kapodistrian University of Athens, 1st Department of Dermatology-Venereology, Andreas Sygros Hospital. Athens, Greece
| | - Kanokvalai Kulthanan
- Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
| | - Alicja Kasperska-Zajac
- European Center for Diagnosis and Treatment of Urticaria/Angioedema (GA(2)LEN UCARE Network), Medical University of Silesia in Katowice, Katowice, Poland
| | - Emek Kocatürk
- Institute of Allergology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany; Institute of Allergology, Fraunhofer Institute for Translational Medicine and Pharmacology (ITMP), Allergology and Immunology, Berlin, Germany; Department of Dermatology, Koç University School of Medicine, Istanbul, Turkey
| | - Michael Makris
- Allergy Unit "D. Kalogeromitros", 2nd Department, Dermatology and Venereology, National and Kapodistrian University of Athens, "Attikon" University Hospital, Athens, Greece
| | - Pavel Kolkhir
- Institute of Allergology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany; Institute of Allergology, Fraunhofer Institute for Translational Medicine and Pharmacology (ITMP), Allergology and Immunology, Berlin, Germany
| | - Karsten Weller
- Institute of Allergology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany; Institute of Allergology, Fraunhofer Institute for Translational Medicine and Pharmacology (ITMP), Allergology and Immunology, Berlin, Germany
| | - Markus Magerl
- Institute of Allergology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany; Institute of Allergology, Fraunhofer Institute for Translational Medicine and Pharmacology (ITMP), Allergology and Immunology, Berlin, Germany
| | - Marcus Maurer
- Institute of Allergology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany; Institute of Allergology, Fraunhofer Institute for Translational Medicine and Pharmacology (ITMP), Allergology and Immunology, Berlin, Germany.
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20
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Lyons JJ, Farkas H, Germenis AE, Rijavec M, Smith TD, Valent P. Genetic Variants Leading to Urticaria and Angioedema and Associated Biomarkers. THE JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY. IN PRACTICE 2023; 11:2286-2301. [PMID: 37263349 PMCID: PMC11854852 DOI: 10.1016/j.jaip.2023.05.031] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Subscribe] [Scholar Register] [Received: 01/13/2023] [Revised: 04/20/2023] [Accepted: 05/15/2023] [Indexed: 06/03/2023]
Abstract
Advances in next generation sequencing technologies, as well as their expanded accessibility and clinical use over the past 2 decades, have led to an exponential increase in the number of identified single gene disorders. Among these are primary atopic disorders-inborn errors of immunity resulting in severe allergic phenotypes as a primary presenting feature. Two cardinal aspects of type I immediate hypersensitivity allergic reactions are hives and angioedema. Mast cells (MCs) are frequent primary drivers of these symptoms, but other cells have also been implicated. Even where MC degranulation is believed to be the cause, mediator-induced symptoms may greatly vary among individuals. Angioedema-particularly in the absence of hives-may also be caused by hereditary angioedema conditions resulting from aberrant regulation of contact system activation and excessive bradykinin generation or impairment of vascular integrity. In these patients, swelling can affect unpredictable locations and fail to respond to MC-directed therapies. Genetic variants have helped delineate key pathways in the etiology of urticaria and nonatopic angioedema and led to the development of targeted therapies. Herein, we describe the currently known inherited and acquired genetic causes for these conditions, highlight specific features in their clinical presentations, and discuss the benefits and limitations of biomarkers that can help distinguish them.
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Affiliation(s)
- Jonathan J Lyons
- Translational Allergic Immunopathology Unit, Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Md.
| | - Henriette Farkas
- Department of Internal Medicine and Haematology, Hungarian Angioedema Center of Reference and Excellence, Semmelweis University, Budapest, Hungary
| | - Anastasios E Germenis
- Department of Immunology and Histocompatibility, School of Medicine, University of Thessaly, Larissa, Greece
| | - Matija Rijavec
- University Clinic of Respiratory and Allergic Diseases Golnik, Golnik, Slovenia; Biotechnical Faculty, University of Ljubljana, Ljubljana, Slovenia
| | - Tukisa D Smith
- Division of Rheumatology, Allergy and Immunology, University of California San Diego, La Jolla, Calif
| | - Peter Valent
- Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria; Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria
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21
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Armstrong AW, Soong W, Bernstein JA. Chronic Spontaneous Urticaria: How to Measure It and the Need to Define Treatment Success. Dermatol Ther (Heidelb) 2023:10.1007/s13555-023-00955-7. [PMID: 37354293 PMCID: PMC10366057 DOI: 10.1007/s13555-023-00955-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/07/2023] [Accepted: 06/01/2023] [Indexed: 06/26/2023] Open
Abstract
Chronic spontaneous urticaria (CSU) is a complex skin disease characterized by the spontaneous appearance of wheals, angioedema, or both, for more than 6 weeks. Many patients experience a relapsing-remitting disease course for years. Owing to the unpredictability of wheal recurrence and the severity of pruritis, patients suffer considerable impairment in their quality of life. Physicians face multiple challenges, not least of which is a lack of clear guidance on what constitutes "treatment success". There is a lack of awareness of which measures should be used to best assess the various aspects of CSU, including disease activity, disease control, and quality of life-which themselves each comprise multiple components-and how to apply the results of each score to treatment decision-making. Although the overarching aim of treatment is for patients to be completely free of signs and symptoms of CSU, a more realistic definition of "treatment success" is needed to guide ongoing, long-term disease management for each individual patient. In this review, we consider what lessons can be learned from the current evidence base to provide further direction toward a universal definition of "treatment success".
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Affiliation(s)
- April W Armstrong
- Department of Dermatology, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.
| | - Weily Soong
- Alabama Allergy and Asthma Center and Clinical Research Center of Alabama, Birmingham, AL, USA
| | - Jonathan A Bernstein
- Division of Immunology/Allergy Section, Department of Internal Medicine, The University of Cincinnati College of Medicine, Cincinnati, OH, USA
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22
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Bernstein JA, Giménez-Arnau A, Maurer M, Staubach P, Barbier N, Hua E, Severin T, Laires PA, Balp MM. Why a Complete Response Is the Treatment Aim in Chronic Spontaneous Urticaria. J Clin Med 2023; 12:jcm12103561. [PMID: 37240667 DOI: 10.3390/jcm12103561] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/13/2023] [Revised: 05/12/2023] [Accepted: 05/14/2023] [Indexed: 05/28/2023] Open
Abstract
This study investigated the association between urticaria activity and health-related quality of life (HRQoL). Patient evaluations from the ligelizumab Phase 2b clinical trial (N = 382) were pooled (NCT02477332). Daily patient diaries assessed urticaria activity, sleep and activity interference, the dermatology life quality index (DLQI), and work productivity and activity impairment-chronic urticaria (WPAI-CU). The number of DLQI scores, weekly sleep interference scores (SIS7), weekly activity interference scores (AIS7), and overall work impairment (OWI) evaluations with a complete response per weekly urticaria activity score (UAS7) using bands (0, 1-6, 7-15, 16-27, and 28-42) were reported. Over 50% of the patients had a mean DLQI of > 10 at baseline, indicating a significant effect of chronic spontaneous urticaria (CSU) on their HRQoL. Complete response (UAS7 = 0) evaluations corresponded with no impacts on other patient-reported outcomes. In total, 91.1% of UAS7 = 0 evaluations corresponded to DLQI scores of 0-1, 99.7% to SIS7 scores of 0, 99.7% to AIS7 scores of 0, and 85.3% to OWI scores of 0. This was significantly different compared with the UAS7 = 1-6 evaluations (61.9%, 68.5%, 67.7%, and 65.4%, respectively; p < 0.0001). Complete responses to treatment were associated with no impairments on the dermatology-QoL, no interferences with sleep and activity, and significantly improved capacities to work compared to patients who continued to have signs and symptoms, even for those with minimal disease activity.
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Affiliation(s)
- Jonathan A Bernstein
- Division of Rheumatology, Bernstein Allergy Group and Clinical Research Center, College of Medicine, University of Cincinnati, Cincinnati, OH 45267, USA
| | - Ana Giménez-Arnau
- Dermatology Department, Hospital del Mar, IMIM, Universitat Pompeu Fabra, 08005 Barcelona, Spain
| | - Marcus Maurer
- Institute of Allergology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, 12203 Berlin, Germany
- Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Allergology and Immunology, 12203 Berlin, Germany
| | - Petra Staubach
- Department of Dermatology, University Medical Center Mainz, 55131 Mainz, Germany
| | | | - Eva Hua
- China Novartis Institutes for Biomedical Research Co., Ltd., Shanghai 201203, China
| | | | - Pedro A Laires
- Novartis Pharma AG, 4002 Basel, Switzerland
- National School of Public Health, Public Health Research Center, Universidade NOVA de Lisboa, 1099-085 Lisbon, Portugal
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23
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Terhorst-Molawi D, Fox L, Siebenhaar F, Metz M, Maurer M. Stepping Down Treatment in Chronic Spontaneous Urticaria: What We Know and What We Don't Know. Am J Clin Dermatol 2023; 24:397-404. [PMID: 36810982 DOI: 10.1007/s40257-023-00761-z] [Citation(s) in RCA: 12] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/24/2023] [Indexed: 02/23/2023]
Abstract
In chronic spontaneous urticaria (CSU), wheals, angioedema, or both appear spontaneously for > 6 weeks. Current recommended treatment options for urticaria target mast cell mediators such as histamine, or activators, such as autoantibodies. The goal of CSU treatment is to treat the disease until it is gone as effectively and safely as possible. As no cure is available for CSU as of now, the treatment is aimed at continuously suppressing disease activity, with complete control of the disease and a normalization of quality of life. To achieve this, pharmacological treatment should be continued until no longer needed. Treatment of CSU should follow the basic principles of treating as much as needed and as little as possible taking into consideration that the activity of the disease may vary. Since CSU is a disease with spontaneous remission, it is hard to tell, in patients with complete control and no signs or symptoms, when medication is no longer needed. The current international guideline for urticaria suggests that the treatment can be stepped down once a patient is free of signs and symptoms. Other reasons for stepping down the treatment of CSU patients include safety concerns or issues, pregnancy or wanting to become pregnant, and economic factors. As of now, it is unclear over which period, with what intervals and with which dosages CSU treatment should be stepped down. Guidance on this is needed for all recommended therapies: (i) standard-dosed second-generation H1-antihistamine (sgAH), (ii) higher than standard-dosed sgAH, (iii) standard-dosed omalizumab, (iv) higher than standard-dosed omalizumab, and (v) cyclosporine. However, there is a lack of controlled trials on the step down and discontinuation of these treatments. Here, we aim to provide a summary of what is known and what needs to be investigated in further studies, based on our own experience and real-world evidence.
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Affiliation(s)
- Dorothea Terhorst-Molawi
- Institute of Allergology, Charité, Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Paul-Ehrlich-Haus, Hindenburgdamm 27, 12203, Berlin, Germany
- Fraunhofer Institute for Translational Medicine and Pharmacology (ITMP), Allergology and Immunology, Berlin, Germany
| | - Lena Fox
- Institute of Allergology, Charité, Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Paul-Ehrlich-Haus, Hindenburgdamm 27, 12203, Berlin, Germany
- Fraunhofer Institute for Translational Medicine and Pharmacology (ITMP), Allergology and Immunology, Berlin, Germany
| | - Frank Siebenhaar
- Institute of Allergology, Charité, Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Paul-Ehrlich-Haus, Hindenburgdamm 27, 12203, Berlin, Germany
- Fraunhofer Institute for Translational Medicine and Pharmacology (ITMP), Allergology and Immunology, Berlin, Germany
| | - Martin Metz
- Institute of Allergology, Charité, Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Paul-Ehrlich-Haus, Hindenburgdamm 27, 12203, Berlin, Germany
- Fraunhofer Institute for Translational Medicine and Pharmacology (ITMP), Allergology and Immunology, Berlin, Germany
| | - Marcus Maurer
- Institute of Allergology, Charité, Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Paul-Ehrlich-Haus, Hindenburgdamm 27, 12203, Berlin, Germany.
- Fraunhofer Institute for Translational Medicine and Pharmacology (ITMP), Allergology and Immunology, Berlin, Germany.
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24
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Özçeker D, Can PK, Terzi Ö, Ornek SA, Değirmentepe EN, Kızıltac K, Sarac E, Kocatürk E. Differences between adult and pediatric chronic spontaneous urticaria from a cohort of 751 patients: Clinical features, associated conditions and indicators of treatment response. Pediatr Allergy Immunol 2023; 34:e13925. [PMID: 36825740 DOI: 10.1111/pai.13925] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/01/2022] [Revised: 01/27/2023] [Accepted: 02/06/2023] [Indexed: 02/25/2023]
Abstract
BACKGROUND Chronic spontaneous urticaria (CSU) is a common disease both in the pediatric and in the adult population. However, there are differences between the two patient populations with respect to etiological factors, comorbidities, and treatment responses. Our aim was to determine differences between pediatric and adult CSU in terms of clinical characteristics, laboratory parameters, comorbidities, response to treatment, and indicators of response. METHODS A retrospective analysis of CSU patients was performed. Data regarding differences between pediatric and adult CSU patients were analyzed. Indicators of treatment response were determined separately in both pediatric and adult patients. RESULTS Of 751 CSU patients (162 pediatrics and 589 adults), female dominancy (48.8% vs. 69.6%) and rate of angioedema (19.1% vs. 59.8%) were lower, and disease duration (5 months vs. 12 months) was shorter in pediatric patients. Anti-TPO positivity (24.7% vs. 9%), elevated CRP (46.5% vs. 11.1%), eosinopenia (38.5% vs. 18.1%), and skin prick test positivity (39.3% vs. 28.8%) were significantly more frequent in adult patients. Response to antihistamines was higher in the pediatric group, and only 7% used omalizumab versus 20.8% in the adults. The comparisons were also performed between <12-year and ≥12-year patients and yielded similar results. CONCLUSION Pediatric CSU shows distinct characteristics such as lower incidence of angioedema and antithyroid antibodies, and it responds better to antihistamines. These suggest that CSU becomes more severe and refractory in adolescents and adults. Adolescent CSU shows features similar to adult CSU rather than pediatric CSU.
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Affiliation(s)
- Deniz Özçeker
- Department of Pediatric Immunology and Allergy, SBU Istanbul Cemil Tascioglu City Hospital, Istanbul, Turkey
| | - Pelin Kuteyla Can
- Faculty of Medicine, Department of Dermatology, Bahcesehir Univercity, Istanbul, Turkey
| | - Özlem Terzi
- Department of Public Health, 19 Mayıs University, Samsun, Turkey
| | - Sinem Ayşe Ornek
- Department of Dermatology, Health Sciences University Diskapi Yildirim Beyazit Training and Research Hospital, Ankara, Turkey
| | - Ece Nur Değirmentepe
- Department of Dermatology, Sakarya Training and Research Hospital, Sakarya, Turkey
| | - Kübra Kızıltac
- Department of Dermatology, Medical Park Gaziosmanpaşa Hospital, Istinye University, Istanbul, Turkey
| | - Esra Sarac
- Department of Dermatology, Koc University School of Medicine, Istanbul, Turkey
| | - Emek Kocatürk
- Department of Dermatology, Koc University School of Medicine, Istanbul, Turkey.,Institute of Allergology, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charité - Universitätsmedizin Berlin, Berlin, Germany.,Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Allergology and Immunology, Berlin, Germany
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Pluta-Kubicz M, Brzoza Z. Chronic Urticaria-Pathogenesis, Diagnostics, Therapy and Influence of Coexisting Angioedema. J Clin Med 2023; 12:jcm12020688. [PMID: 36675617 PMCID: PMC9862754 DOI: 10.3390/jcm12020688] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/20/2022] [Accepted: 01/11/2023] [Indexed: 01/18/2023] Open
Abstract
Urticaria is one of the most frequent dermatological diseases and it usually occurs in paroxysmal, recurrent form [...].
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Affiliation(s)
- Marzena Pluta-Kubicz
- Department of Internal Diseases, Allergology and Clinical Immunology, Faculty of Medical Sciences in Zabrze, Medical University of Silesia, 40-055 Katowice, Poland
| | - Zenon Brzoza
- Department of Internal Diseases, Allergology, Endocrinology and Gastroenterology, Institute of Medical Sciences, University of Opole, 45-401 Opole, Poland
- Correspondence:
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26
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Wiuff AC, Knudsgaard Wiis MA, Heilskov S, Frølunde AS, Sørensen JA, Deleuran M, Thomsen SF, Vestergaard C. The impact of home treatment and self-administration of omalizumab on chronic urticaria. World Allergy Organ J 2022; 15:100725. [PMID: 36531648 PMCID: PMC9731880 DOI: 10.1016/j.waojou.2022.100725] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/15/2022] [Revised: 11/01/2022] [Accepted: 11/14/2022] [Indexed: 12/12/2022] Open
Abstract
Background Patients suffering from Chronic Spontaneous Urticaria (CSU) may be recalcitrant to treatment with high dose second-generation antihistamines. These patients are, according to international guidelines, eligible to treatment with omalizumab. Treatment with omalizumab has proven to be very effective for this group of patients. Until 2018, when the self-administration of omalizumab was approved, patients have had to visit an outpatient clinic to receive the treatment. Methods In this study we performed a survey among 75 CSU patients who had changed their treatment from administration at an outpatient clinic to self-administration at home. Results None of the patients experienced decreased effect of the treatment with omalizumab, whereas 41.4% reported improved effect of the treatment during self-administration.The most common reported benefits for the patients were decreased travel time and the ability to choose dosing time at the day of treatment, whereas the most reported concern was fear of performing the injection. Conclusions Patients are very content with self-administration of omalizumab. There has been no decreased effect of the treatment, and as discussed in this paper, there might be other societal benefits from self-administration. Thus, self-administration at home of omalizumab should be standard of care when using this treatment.
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Affiliation(s)
| | | | - Sofine Heilskov
- Department of Dermatology, Aarhus University Hospital, Aarhus, Denmark
| | | | | | - Mette Deleuran
- Department of Dermatology, Aarhus University Hospital, Aarhus, Denmark
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Ayse Ornek S, Orcen C, Church MK, Kocaturk E. An evaluation of remission rates with first and second line treatments and indicators of antihistamine refractoriness in chronic urticaria. Int Immunopharmacol 2022; 112:109198. [PMID: 36115277 DOI: 10.1016/j.intimp.2022.109198] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/10/2022] [Revised: 08/22/2022] [Accepted: 08/23/2022] [Indexed: 11/29/2022]
Abstract
BACKGROUND Guidelines recommend standard doses of antihistamines as first-line, and updosing of antihistamines as second-line treatment for the management of chronic urticaria (CU). However, remission rates with different types of first- and second-line treatments and indicators of antihistamine response are largely lacking in the literature. OBJECTIVES To examine response rates to first- and second-line treatments in CU, and to identify patient characteristics that can predict antihistamine treatment outcomes. METHODS We retrospectively analyzed treatment outcomes of 657 CU (556 chronic spontaneous urticaria (CSU), 101 chronic inducible urticaria (CIndU)) patients who had at least 3-months of follow-up data. RESULTS A standard dose of second generation antihistamines (sgAH) was effective in 43.1 % of the patients. An additional 28.8 % of patients were in remission with second-line treatments. Among patients whose disease was in remission with a standard dose of sgAHs, 14.8 % benefited from switching from their current sgAH to another sgAH. Updosing sgAHs, combination of two different sgAHs, sgAH and first generation H1-antihistamine combination, and sgAH and leukotriene receptor antagonist combination provided remission in 38.3 %, 35.8 %, 37.5 % and 25 % of patients who were given these treatments, respectively. Baseline UCT score ≤ 4, emergency referral and family history of CSU were found to be risk factors for antihistamine refractoriness in patients with CSU. CONCLUSIONS A step-wise approach to the management of CU is practical as more patients respond to treatment at each step. The presence of baseline UCT score ≤ 4, emergency referral and family history of CSU might be helpful to determine patients who require third-line treatments in advance.
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Affiliation(s)
- Sinem Ayse Ornek
- Department of Dermatology, Health Sciences University Diskapi Yildirim Beyazit Training and Research Hospital, Ankara, Turkey
| | - Cihan Orcen
- Department of Allergy Immunology, Health Science University Derince Training and Research Hospital, Kocaeli, Turkey
| | - Martin K Church
- Institute for Allergology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany
| | - Emek Kocaturk
- Institute for Allergology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany; Department of Dermatology, Koc University School of Medicine, Istanbul, Turkey; Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Allergology and Immunology, Berlin, Germany.
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Rodríguez-Garijo N, Sabaté-Brescó M, Azofra J, Baeza ML, Donado CD, Gaig P, Guilarte M, Herrera-Lasso V, Labrador-Horrillo M, Sala-Cunill A, Veleiro B, Gil MP, Ferrer M. Angioedema severity and impact on quality of life: Chronic histaminergic angioedema versus chronic spontaneous urticaria. THE JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY. IN PRACTICE 2022; 10:3039-3043.e3. [PMID: 35934307 DOI: 10.1016/j.jaip.2022.07.011] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 11/17/2021] [Revised: 06/29/2022] [Accepted: 07/11/2022] [Indexed: 06/15/2023]
Affiliation(s)
| | - Marina Sabaté-Brescó
- Department of Allergy and Clinical Immunology, Clinica Universidad de Navarra, Pamplona, Spain; Instituto de Investigación Sanitaria de Navarra, Pamplona, Spain; RETIC de Asma, Reacciones Adversas y Alérgicas, Spain
| | - Julián Azofra
- Allergy Section, Hospital Universitario Central de Asturias, Oviedo, Spain
| | - Maria Luisa Baeza
- RETIC de Asma, Reacciones Adversas y Alérgicas, Spain; Allergy Service, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Biomedical Research Network on Rare Diseases (CIBERER)-U761, Gregorio Marañón Health Research Institute, Madrid, Spain
| | - Camen Diaz Donado
- Allergy Section, Hospital Universitario Central de Asturias, Oviedo, Spain
| | - Pere Gaig
- Allergy Section, Hospital Universitari Joan XXIII de Tarragona, Institut d'Investigació Sanitària Pere Virgili, Universitat Rovira i Virgili, Tarragona, Spain
| | - Mar Guilarte
- RETIC de Asma, Reacciones Adversas y Alérgicas, Spain; Allergy Section, Internal Medicine Department, Hospital Universitari Vall d'Hebron, Barcelona, Spain
| | - Valeria Herrera-Lasso
- Allergy Section, Hospital Universitari Joan XXIII de Tarragona, Institut d'Investigació Sanitària Pere Virgili, Universitat Rovira i Virgili, Tarragona, Spain
| | - Moisés Labrador-Horrillo
- RETIC de Asma, Reacciones Adversas y Alérgicas, Spain; Allergy Section, Internal Medicine Department, Hospital Universitari Vall d'Hebron, Barcelona, Spain
| | - Anna Sala-Cunill
- RETIC de Asma, Reacciones Adversas y Alérgicas, Spain; Allergy Section, Internal Medicine Department, Hospital Universitari Vall d'Hebron, Barcelona, Spain
| | - Beatriz Veleiro
- Allergy Department, Complexo Hospitalario Universitario de A Coruña, A Coruña, Spain
| | - Maria Pilar Gil
- Department of Dermatology, Clinica Universidad de Navarra, Pamplona, Spain
| | - Marta Ferrer
- Department of Allergy and Clinical Immunology, Clinica Universidad de Navarra, Pamplona, Spain; Instituto de Investigación Sanitaria de Navarra, Pamplona, Spain; RETIC de Asma, Reacciones Adversas y Alérgicas, Spain.
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Oztop N, Demir S, Unal D, Beyaz S, Terzioglu K, Olgac M, Gelincik A. Predictive factors of recurrence after omalizumab cessation in the elderly with urticaria: A real-life study. Allergy Asthma Proc 2022; 43:519-528. [PMID: 36335414 DOI: 10.2500/aap.2022.43.220068] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/09/2022]
Abstract
Background: There are no well-defined data that help predict the recurrence risk of urticaria after omalizumab cessation in elderly patients with chronic spontaneous urticaria (CSU). Objective: We aimed to evaluate the effectiveness and safety of omalizumab and to determine the possible predictive factors for recurrence after omalizumab cessation in the elderly with CSU. Methods: A total of 193 patients with CSU treated with omalizumab were included and divided into two groups according to age: group 1, ages 18-64 years (n = 127), and group 2, ages ≥ 65 years (n = 66). Demographics, clinical features, immunoglobulin G (IgG) anti-thyroid peroxidase antibody (anti-TPO), serum total IgE were analyzed. The IgG anti-TPO/total IgE ratio was calculated. Pretreatment 7-day urticaria activity scores, medication scores, and urticaria control test results were compared with those after treatment periods. Adverse effects were also evaluated. Results: The most common adverse effect of omalizumab treatment was injection-site reactions (4.7%) in both groups. Omalizumab was ceased after 24 weeks in 40.9% and in 73.1% in group 1 and group 2, respectively (p < 0.001). CSU recurred after omalizumab discontinuation in 9 and 15 patients in group 1 and in group 2, respectively (p < 0.001). The median baseline IgG anti-TPO was higher in patients with recurrent CSU in group 2 than in those in group 1 (p = 0.002). In group 2, the cutoff values of IgG anti-TPO and the IgG anti-TPO/total IgE ratio were 54.83 IU/mL and 0.45 for recurrence, respectively. Conclusion: Omalizumab is effective and safe in elderly patients with CSU. The serum baseline IgG anti-TPO level and the IgG anti-TPO/total IgE ratio could serve as predictors of recurrence in CSU after omalizumab cessation in elderly patients.
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Affiliation(s)
- Nida Oztop
- From the Division of Immunology and Allergic Diseases, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey
| | - Semra Demir
- From the Division of Immunology and Allergic Diseases, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey
| | - Derya Unal
- From the Division of Immunology and Allergic Diseases, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey
| | - Sengul Beyaz
- Department of Adult Allergy and Clinical Immunology, Ankara City Hospital, Ankara, Turkey
| | - Kadriye Terzioglu
- Department of Adult Allergy and Clinical Immunology, SBÜ Kartal Education and Training Hospital, Istanbul, Turkey, and
| | - Muge Olgac
- Department of Adult Allergy and Clinical Immunology, SBU Sisli Hamidiye Etfal Education and Training Hospital, Istanbul, Turkey
| | - Aslı Gelincik
- From the Division of Immunology and Allergic Diseases, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey
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Metz M, Bernstein JA, Giménez-Arnau AM, Hide M, Maurer M, Sitz K, Soong W, Sussman G, Hua E, Barve A, Barbier N, Balp MM, Severin T. Ligelizumab improves angioedema, disease severity and quality-of-life in patients with chronic spontaneous urticaria. World Allergy Organ J 2022; 15:100716. [PMID: 36440464 PMCID: PMC9672946 DOI: 10.1016/j.waojou.2022.100716] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/30/2022] [Revised: 09/07/2022] [Accepted: 10/19/2022] [Indexed: 11/17/2022] Open
Abstract
Background Disease burden is particularly high in Chronic Spontaneous Urticaria (CSU) patients with angioedema, and patients whose signs and symptoms are inadequately controlled by H1-antihistamines need new treatment options. Here we report an exploratory analysis, from the ligelizumab Phase 2b study, investigating angioedema occurrence in patients with CSU and describe the changes in angioedema following treatment with ligelizumab, omalizumab, or placebo. Methods Data from the ligelizumab Phase 2b core (ligelizumab 72 mg, 240 mg, omalizumab 300 mg and placebo) and extension study (ligelizumab 240 mg) were used. Changes in Weekly Angioedema Activity Score (AAS7), the Dermatology Life Quality Index (DLQI), and Weekly Urticaria Activity Score (UAS7) among each time point were analyzed for each treatment arm. Results From a total of 297 patients analyzed, 165 (55.6%) reported angioedema occurrence at baseline, with mean AAS7 ranging 30.6—42.2 across treatment arms. At Week 12 of the core study 87.5%, 84.6%, 75.0%, and 61.0% of patients were angioedema free for ligelizumab 72 mg, 240 mg, omalizumab 300 mg, and placebo arms, respectively. In CSU patients with angioedema at baseline, the largest change from baseline in AAS7 score was observed with ligelizumab 72 mg (−31.9) at week 16 in the core study. Patients with angioedema had a higher mean DLQI at baseline (14.9—16.1) vs. patients without angioedema (10.6—12.0). In patients with angioedema, low AAS7 was significantly associated with complete response on UAS7 (UAS7 = 0) and complete normalization of DLQI (DLQI 0—1). Conclusion In the Phase 2b study, ligelizumab effectively reduced angioedema and urticaria symptoms, and improve health related quality of life in patients with moderate-to-severe CSU. Clinicaltrails.gov NCT number NCT02477332; NCT02649218.
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Kan AKC, Wong TTH, Chiang V, Lau CS, Li PH. Chronic Spontaneous Urticaria in Hong Kong: Clinical Characteristics, Real-World Practice and Implications for COVID-19 Vaccination. ALLERGY, ASTHMA & IMMUNOLOGY RESEARCH 2022; 15:32-42. [PMID: 36693356 PMCID: PMC9880305 DOI: 10.4168/aair.2023.15.1.32] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 05/26/2022] [Revised: 07/14/2022] [Accepted: 07/26/2022] [Indexed: 01/19/2023]
Abstract
PURPOSE The real-world management and clinical characteristics of chronic spontaneous urticaria (CSU) in Hong Kong and its implications for coronavirus disease 2019 (COVID-19) vaccination are unknown. We investigated the clinical characteristics of patients with CSU and the role of an immunologist-led Urticaria Clinic as well as the impact of CSU on COVID-19 vaccine uptake in Hong Kong. METHODS Longitudinal clinical data of 257 CSU patients were collected and analyzed. Association analyses were performed to identify the relationships between variables and factors associated with COVID-19 vaccine uptake. RESULTS After the immunologist review, the Weekly Urticaria Activity Score (UAS7) was significantly lower than baseline (median: 0.00 vs. 12.0, P < 0.001). Changes in UAS7 were significantly greater among patients with baseline UAS7 ≥ 16 compared to those with UAS7 < 16 (median: -24.0 vs. -2.00, P < 0.001). CSU patients had lower COVID-19 vaccination rates than the general population with only 176 (68.5%) and 165 (65.0%) receiving at least one dose and 2 doses of vaccination, respectively. The presence of concomitant suspected drug allergy was associated with lower COVID-19 vaccine uptake (odds ratio [OR], 0.47; P = 0.010), while regular pharmacological treatment was associated with higher COVID-19 vaccine uptake among CSU patients (OR, 3.79; P = 0.010). CONCLUSIONS A dedicated immunologist-led Urticaria Clinic may effectively improve CSU management and outcomes in Hong Kong.
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Affiliation(s)
- Andy Ka Chun Kan
- Division of Rheumatology and Clinical Immunology, Department of Medicine, The University of Hong Kong, Hong Kong
| | - Thomas Tsz Hang Wong
- Division of Rheumatology and Clinical Immunology, Department of Medicine, The University of Hong Kong, Hong Kong
| | - Valerie Chiang
- Division of Clinical Immunology, Department of Pathology, Queen Mary Hospital, Hong Kong
| | - Chak Sing Lau
- Division of Rheumatology and Clinical Immunology, Department of Medicine, The University of Hong Kong, Hong Kong
| | - Philip Hei Li
- Division of Rheumatology and Clinical Immunology, Department of Medicine, The University of Hong Kong, Hong Kong
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Prospective analysis of clinical evolution in chronic urticaria: Persistence, remission, recurrence, and pruritus alone. World Allergy Organ J 2022; 15:100705. [PMID: 36267098 PMCID: PMC9554810 DOI: 10.1016/j.waojou.2022.100705] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/25/2022] [Revised: 06/25/2022] [Accepted: 09/07/2022] [Indexed: 11/21/2022] Open
Abstract
Background Population and study's methodology heterogenicity became clinical evolution of chronic spontaneous urticaria (CSU) highly variable. Objective In a prospective cohort, we evaluated the different pathways of clinical evolution of CSU and identified possible risk factors. Methods A total of 685 CSU patients (>12 years) were prospectively followed over 5 years. Diagnosis and follow-up of urticaria were based on medical evaluation and photographic records. Remission was defined as at least 6 months without symptoms (hives, angioedema, or pruritus) and medication. The follow-up included at least 2 visits per year, with photographic registration and clinical evaluation. Predefined clinical and paraclinical variables were included in the regression analyses. Results We identified four clinical evolution pathways; The cumulative prevalence of remission at 5 years was 59.1%, recurrence was 17.1%, persistence was 11.6%, and chronic pruritus without hives or angioedema was 12.2%. The probability of persistence increased with hypothyroidism diagnosis (HR 0.425, 95% CI 0.290-0.621) and each point in the UAS7 (HR 0.931 95% CI 0.918-0.945). Conclusion Chronic urticaria has different evolutions. Disease activity and hypothyroidism predict persistence and remission. Recurrence and chronic pruritus phenotypes require further study to evaluate their causality and prognosis.
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Abstract
Urticaria is an inflammatory skin disorder that affects up to 20% of the world population at some point during their life. It presents with wheals, angioedema or both due to activation and degranulation of skin mast cells and the release of histamine and other mediators. Most cases of urticaria are acute urticaria, which lasts ≤6 weeks and can be associated with infections or intake of drugs or foods. Chronic urticaria (CU) is either spontaneous or inducible, lasts >6 weeks and persists for >1 year in most patients. CU greatly affects patient quality of life, and is linked to psychiatric comorbidities and high healthcare costs. In contrast to chronic spontaneous urticaria (CSU), chronic inducible urticaria (CIndU) has definite and subtype-specific triggers that induce signs and symptoms. The pathogenesis of CSU consists of several interlinked events involving autoantibodies, complement and coagulation. The diagnosis of urticaria is clinical, but several tests can be performed to exclude differential diagnoses and identify underlying causes in CSU or triggers in CIndU. Current urticaria treatment aims at complete response, with a stepwise approach using second-generation H1 antihistamines, omalizumab and cyclosporine. Novel treatment approaches centre on targeting mediators, signalling pathways and receptors of mast cells and other immune cells. Further research should focus on defining disease endotypes and their biomarkers, identifying new treatment targets and developing improved therapies.
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Affiliation(s)
- Pavel Kolkhir
- Urticaria Center of Reference and Excellence (UCARE), Institute of Allergology, Charité - Universitätsmedizin Berlin, Berlin, Germany.
- Allergology and Immunology, Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Berlin, Germany.
| | - Ana M Giménez-Arnau
- Urticaria Center of Reference and Excellence (UCARE), Department of Dermatology, Hospital del Mar, Institut Mar d'Investigacions Mediques, Universitat Autònoma, Barcelona, Spain
| | - Kanokvalai Kulthanan
- Urticaria Center of Reference and Excellence (UCARE), Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
| | - Jonny Peter
- Urticaria Center of Reference and Excellence (UCARE), Division of Allergy and Clinical Immunology, Department of Medicine, University of Cape Town, Cape Town, South Africa
- Urticaria Center of Reference and Excellence (UCARE), Allergy and Immunology Unit, University of Cape Town, Lung Institute, Cape Town, South Africa
| | - Martin Metz
- Urticaria Center of Reference and Excellence (UCARE), Institute of Allergology, Charité - Universitätsmedizin Berlin, Berlin, Germany
- Allergology and Immunology, Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Berlin, Germany
| | - Marcus Maurer
- Urticaria Center of Reference and Excellence (UCARE), Institute of Allergology, Charité - Universitätsmedizin Berlin, Berlin, Germany.
- Allergology and Immunology, Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Berlin, Germany.
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Ye YM, Koh YI, Choi JH, Kim MA, Park JW, Kim TB, Nam YH, Chang YS, Park HS. The burden of symptomatic patients with chronic spontaneous urticaria: a real-world study in Korea. Korean J Intern Med 2022; 37:1050-1060. [PMID: 36068718 PMCID: PMC9449199 DOI: 10.3904/kjim.2022.078] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/23/2022] [Revised: 04/15/2022] [Accepted: 05/09/2022] [Indexed: 11/30/2022] Open
Abstract
BACKGROUND/AIMS Chronic spontaneous urticaria (CSU) poses a considerable burden both on the quality of life (QoL) of individual patients and on healthcare systems. Real‑world data evaluating the disease burden of CSU are limited in this country. This study evaluated the disease burden and healthcare resource utilization (HRU) among symptomatic CSU patients. METHODS This multicenter, noninterventional, retrospective, and cross-sectional study assessed CSU patients symptomatic for more than 6 months despite step-wise H1-antihistamine medications. Primary outcomes included Urticaria Activity Score over 7 days (UAS7) and Chronic Urticaria QoL scale (CU-QoL). Secondary outcomes included EuroQol 5-Dimension 5-Level (EQ-5D-5L), Dermatology Life Quality Index (DLQI), association of disease activity with QoL, medications used for the past 6 months, and HRU. RESULTS Five hundred patients with CSU were enrolled. Mean disease duration was 3.7 years. Based on UAS7, 22.2% of patients were in well-controlled status and 31.2%, 28.4%, and 18.2% of them had mild, moderate, and severe disease, respectively. Mean CU-QoL and DLQI scores were 57.5 ± 29.7 and 10.2 ± 7.6, respectively, while the EQ-5D-5L utility score was 0.8 ± 0.2. H1-antihistamines were prescribed to 95% of patients, while omalizumab was prescribed to 33% of patients. Most patients (98%) had outpatient visits in the past 6 months. Negative correlations were noted between UAS7 and CU-QoL, EQ-5D-5L, EQ-5D-5L visual analog scale scores, but a positive correlation was noted with DLQI score (p < 0.001 for all). The number of outpatient department visits increased with disease activity (p = 0.001). CONCLUSION CSU affects QoL, leading to increased HRU, particularly in patients with severe disease.
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Affiliation(s)
- Young-Min Ye
- Department of Allergy and Clinical Immunology, Ajou University School of Medicine, Suwon, Korea
| | - Young-Il Koh
- Department of Allergy, Asthma and Clinical Immunology, Chonnam National University Hospital, Chonnam National University Medical School, Gwangju, Korea
| | - Jeong-Hee Choi
- Department of Pulmonology and Allergy, Hallym University Dongtan Sacred Heart Hospital, Hwaseong, Korea
| | - Mi-Ae Kim
- Department of Pulmonology, Allergy and Critical Care Medicine, CHA Bundang Medical Center, CHA University, Seongnam, Korea
| | - Jung-Won Park
- Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
| | - Tae-Bum Kim
- Department of Allergy and Clinical Immunology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Young-Hee Nam
- Department of Internal Medicine, Dong-A University College of Medicine, Busan, Korea
| | - Yoon-Seok Chang
- Department of Internal Medicine, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea
| | - Hae-Sim Park
- Department of Allergy and Clinical Immunology, Ajou University School of Medicine, Suwon, Korea
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Saito R, Takahagi S, Nakano F, Furutani K, Mihara S, Numata T, Kameyoshi Y, Tanaka T, Shindo H, Niimi N, Iwamoto K, Hiragun T, Hiragun M, Tanaka A, Hide M. A survey on subtypes and clinical characteristics of 1061 patients with urticaria in the primary care institutes in Japan. J Dermatol 2022; 49:1255-1262. [PMID: 36028980 DOI: 10.1111/1346-8138.16562] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/01/2022] [Revised: 07/01/2022] [Accepted: 08/11/2022] [Indexed: 11/26/2022]
Abstract
The prevalence of urticaria has been reported mostly in Europe and North America. However, precise information regarding its subtypes and clinical characteristics in primary care practice, especially in Asian countries, are scant. Patients with urticaria and/or angioedema who visited nine primary clinics of accredited dermatologists and allergologists in Japan were recruited from October to November 2020. The information of age, sex, disease duration, urticaria control test (UCT), and concomitant urticaria subtypes were collected. A total of 1061 patients participated. The number of patients was high in the 20 to 50 age groups with a peak in the 40s. The most frequent urticaria subtype was chronic spontaneous urticaria (CSU) followed by dermographism, acute spontaneous urticaria (ASU), angioedema, and cholinergic urticaria (CholU) (66.8%, 22.7%, 18.9%, 14.1% and 5.7% in all patients with urticaria). CSU development increased with age from the 20s to 50s, especially in females. Dermographism had a peak in the 40s. ASU had bimodal peaks in childhood and in the 30s. CholU was common in males in the 10-20s. Most angioedema patients were female with an increase in their 30s. Angioedema was solely present in 14 of 1061 participants (1.3%), while 136 (12.8%) had angioedema concomitant with urticaria. UCT showed poorly controlled urticaria with lower scores in patients with concomitant CSU and other subtypes than in those with CSU alone. Urticaria tends to develop in young to middle-aged females. The most common urticaria subtype is CSU, while the number of patients with CholU is high and that of angioedema is low in Japan.
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Affiliation(s)
- Ryo Saito
- Department of Dermatology, Institute of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan
| | - Shunsuke Takahagi
- Department of Dermatology, Institute of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan
| | - Fumi Nakano
- Department of Dermatology, Institute of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan
| | | | - Shoji Mihara
- Mihara Dermatology/Allergy Clinic, Hiroshima, Japan
| | | | | | | | | | | | | | | | | | - Akio Tanaka
- Department of Dermatology, Institute of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan
| | - Michihiro Hide
- Department of Dermatology, Institute of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan
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Jiménez LF, Castellón EA, Marenco JD, Mejía JM, Rojas CA, Jiménez FT, Coronell L, Osorio-Llanes E, Mendoza-Torres E. Chronic urticaria associated with lung adenocarcinoma — a paraneoplastic manifestation: A case report and literature review. World J Clin Cases 2022; 10:7553-7564. [PMID: 36157992 PMCID: PMC9353901 DOI: 10.12998/wjcc.v10.i21.7553] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/11/2022] [Revised: 04/07/2022] [Accepted: 06/03/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Urticaria is one of the most common causes of emergency room visits. It is defined as an acute inflammatory dermatosis, characterized by localized degranulation of mast cells, with consequent dermal microvascular and formation of edematous and pruritic plaques called hives. Urticaria affects the skin and tissues of the superficial mucosa. Sometimes it is accompanied by angioedema, which is characterized by deeper edema of the dermis and subcutaneous cellular tissue known as the urticarial-angioedema syndrome. About 15%-25% of the general population has suffered at least one type of urticaria at some point during their lifetime and hyperpermeability estimated at 7.6%-16% and has experienced acute urticaria that is usually self-limited and spontaneously resolves without requiring medical attention.
CASE SUMMARY We present the case of a young male patient who was referred to our department with a clinical picture of 4 mo of pruritus associated with hives of variable sizes, irregular borders, with interlesional confluence, that were non-painful, without involvement of the palms and soles of the feet but with a tendency to progression in a generalized manner. He had multiple emergency room visits and poor response to antihistamines and systemic corticosteroids. Imaging studies demonstrated nodules in the lower lingula segment, at the level of the greater fissure and in the anterior contour of the left anterior basal segment associated with parahiliar adenopathies in the absence of findings suggestive of infectious or autoimmune etiology. Segmental lobectomy was performed by thoracoscopy with resection of a lung nodule in the lingula and biopsy of the para-aortic mediastinal ganglion. The histopathological report showed the presence of poorly differentiated invasive adenocarcinoma with a solid morphological and acinar pattern with immunohistochemical description of lung tissue that expresses strong positive and diffuse reaction for thyroid transcription factor 1 (TTF-1) with negativity to P40 for a histopathological diagnosis of malignant epithelial neoplasia with expression of infiltrating adenocarcinoma. Spontaneous chronic urticaria is considered possibly secondary to lung adenocarcinoma.
CONCLUSION Chronic spontaneous urticaria is considered a paraneoplastic dermatosis with a controversial association in the literature. In the presented case, a young patient presented with chronic refractory urticaria and after an exhaustive clinical work-up was found to have a diagnosis of poorly differentiated lung adenocarcinoma with high expression of TTF-1. According to the Curth criteria, the urticaria presented by the patient is related to the oncological diagnosis. In addition, the high expression of TTF-1 documented in this case could be acting as an autoantigen that would cause chronic spontaneous urticaria. Further research evaluating a causal relationship between the TFF-1 protein and urticaria in lung cancer is needed.
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Affiliation(s)
- Luisa Fernanda Jiménez
- Faculty of Health Sciences, Universidad Libre de Colombia, Seccional Barranquilla, Barranquilla 08000, Colombia
| | - Evis Adriana Castellón
- Faculty of Health Sciences, Universidad Libre de Colombia, Seccional Barranquilla, Barranquilla 08000, Colombia
| | - Juan David Marenco
- Faculty of Health Sciences, Universidad Libre de Colombia, Seccional Barranquilla, Barranquilla 08000, Colombia
| | - José Maria Mejía
- Faculty of Health Sciences, Universidad Libre de Colombia, Seccional Barranquilla, Barranquilla 08000, Colombia
| | - Camilo Andrés Rojas
- Faculty of Health Sciences, Universidad Libre de Colombia, Seccional Barranquilla, Barranquilla 08000, Colombia
| | - Franklin Torres Jiménez
- Faculty of Health Sciences, Universidad Libre de Colombia, Seccional Barranquilla, Barranquilla 08000, Colombia
| | - Linda Coronell
- Faculty of Health Sciences, Universidad Libre de Colombia, Seccional Barranquilla, Barranquilla 08000, Colombia
| | - Estefanie Osorio-Llanes
- Faculty of Exact and Natural Sciences, Universidad Libre de Colombia, Seccional Barranquilla, Barranquilla 08000, Colombia
| | - Evelyn Mendoza-Torres
- Faculty of Health Sciences, Universidad Libre De Colombia, Seccional Barranquilla, Barranquilla 08000, Atlántico, Colombia
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Votto M, Achilli G, De Filippo M, Licari A, Marseglia A, Moiraghi A, Di Sabatino A, Marseglia GL. Pediatric chronic spontaneous urticaria: a brief clinician's guide. Expert Rev Clin Immunol 2022; 18:889-899. [PMID: 35833360 DOI: 10.1080/1744666x.2022.2101999] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022]
Abstract
INTRODUCTION Chronic urticaria (CU) appears with daily or intermittent/recurrent wheals with/without angioedema for more than six weeks. When no specific eliciting factors are found, chronic urticaria is defined as spontaneous (CSU). Up to 50% of patients with CSU do not respond to therapy, leading to a prolonged disease course and the need for expensive therapies, impacting the quality of life (QoL) and healthcare resources. AREAS COVERED : Diagnosis of CSU is made when other potential causes of chronic urticaria are excluded. CSU therapy aims to achieve complete control of symptoms and normalization of QoL. Current treatment options for urticaria aim to target mast cell mediators such as histamine, or activators, such as autoantibodies. Guidelines recommend starting with second generation antihistamines (sgAHs) and adding omalizumab therapy if symptoms are not controlled. This review aims to provide a practical guide for CSU in the pediatric population. EXPERT OPINION : Treatment options for pediatric CSU are primarily based on adult data that have been extrapolated for children. Current guidelines should be re-evaluated based on pediatric data, new biological treatments, and the COVID-19 pandemic. Future research is needed to investigate strategies to personalize current treatments and identify potential predictive biomarkers.
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Affiliation(s)
- Martina Votto
- Pediatric Unit, Department of Clinical, Surgical, Diagnostic and Pediatric Sciences, University of Pavia, Italy
| | - Giovanna Achilli
- Department of Internal Medicine, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy
| | - Maria De Filippo
- Pediatric Unit, Department of Clinical, Surgical, Diagnostic and Pediatric Sciences, University of Pavia, Italy.,Pediatric Clinic, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
| | - Amelia Licari
- Pediatric Unit, Department of Clinical, Surgical, Diagnostic and Pediatric Sciences, University of Pavia, Italy.,Pediatric Clinic, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
| | - Alessia Marseglia
- Pediatric Unit, Department of Clinical, Surgical, Diagnostic and Pediatric Sciences, University of Pavia, Italy
| | - Alice Moiraghi
- Pediatric Unit, Department of Clinical, Surgical, Diagnostic and Pediatric Sciences, University of Pavia, Italy
| | - Antonio Di Sabatino
- Department of Internal Medicine, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy
| | - Gian Luigi Marseglia
- Pediatric Unit, Department of Clinical, Surgical, Diagnostic and Pediatric Sciences, University of Pavia, Italy.,Pediatric Clinic, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
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Kocatürk E, Podder I, Zenclussen AC, Kasperska Zajac A, Elieh-Ali-Komi D, Church MK, Maurer M. Urticaria in Pregnancy and Lactation. FRONTIERS IN ALLERGY 2022; 3:892673. [PMID: 35873599 PMCID: PMC9300824 DOI: 10.3389/falgy.2022.892673] [Citation(s) in RCA: 11] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/09/2022] [Accepted: 06/09/2022] [Indexed: 11/13/2022] Open
Abstract
Chronic urticaria (CU) is a mast cell-driven chronic inflammatory disease with a female predominance. Since CU affects mostly females in reproductive age, pregnancy is an important aspect to consider in the context of this disease. Sex hormones affect mast cell (MC) biology, and the hormonal changes that come with pregnancy can modulate the course of chronic inflammatory conditions, and they often do. Also, pregnancy-associated changes in the immune system, including local adaptation of innate and adaptive immune responses and skewing of adaptive immunity toward a Th2/Treg profile have been linked to changes in the course of inflammatory diseases. As of now, little is known about the effects of pregnancy on CU and the outcomes of pregnancy in CU patients. Also, there are no real-life studies to show the safety of urticaria medications during pregnancy. The recent PREG-CU study provided the first insights on this and showed that CU improves during pregnancy in half of the patients, whereas it worsens in one-third; and two of five CU patients experience flare-ups of their CU during pregnancy. The international EAACI/GA2LEN/EuroGuiDerm/APAAACI guideline for urticaria recommends adopting the same management strategy in pregnant and lactating CU patients; starting treatment with standard doses of second-generation (non-sedative) H1 antihistamines, to increase the dose up to 4-folds in case of no response, and to add omalizumab in antihistamine-refractory patients; but also emphasizes the lack of evidence-based information on the safety and efficacy of urticaria treatments during pregnancy. The PREG-CU study assessed treatments and their outcomes during pregnancy. Here, we review the reported effects of sex hormones and pregnancy-specific immunological changes on urticaria, we discuss the impact of pregnancy on urticaria, and we provide information and guidance on the management of urticaria during pregnancy and lactation.
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Affiliation(s)
- Emek Kocatürk
- Department of Dermatology, Koç University School of Medicine, Istanbul, Turkey
- *Correspondence: Emek Kocatürk
| | - Indrashis Podder
- Department of Dermatology, Venereology and Leprosy, College of Medicine and Sagore Dutta Hospital, Kolkata, India
| | - Ana C. Zenclussen
- Department of Environmental Immunology, Helmholtz Centre for Environmental Research (UFZ) and Saxonian Incubator for Clinical Translation (SIKT), University of Leipzig, Leipzig, Germany
| | - Alicja Kasperska Zajac
- European Center for Diagnosis and Treatment of Urticaria/Angioedema (GA2LEN UCARE /ACARE Network), Zabrze, Poland
- Department of Clinical Allergology, Urticaria Center of Medical University of Silesia, Katowice, Poland
| | - Daniel Elieh-Ali-Komi
- Institute of Allergology, Charité – Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany
- Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Allergology and Immunology, Berlin, Germany
| | - Martin K. Church
- Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Allergology and Immunology, Berlin, Germany
| | - Marcus Maurer
- Institute of Allergology, Charité – Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany
- Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Allergology and Immunology, Berlin, Germany
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Oztop N, Beyaz S, Orcen C. Abdominal Obesity and Metabolic Parameters in Chronic Spontaneous Urticaria. HASEKI TIP BÜLTENI 2022. [DOI: 10.4274/haseki.galenos.2022.8399] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/01/2022] Open
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Giménez‐Arnau A, Maurer M, Bernstein J, Staubach P, Barbier N, Hua E, Severin T, Joubert Y, Janocha R, Balp M. Ligelizumab improves sleep interference and disease burden in patients with chronic spontaneous urticaria. Clin Transl Allergy 2022; 12:e12121. [PMID: 35218324 PMCID: PMC8848195 DOI: 10.1002/clt2.12121] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/31/2021] [Revised: 12/27/2021] [Accepted: 01/25/2022] [Indexed: 12/19/2022] Open
Abstract
BACKGROUND Chronic spontaneous urticaria (CSU) negatively impacts patients' sleep, thereby reducing health-related quality of life (HRQoL). Half of patients with inadequately controlled CSU report sleep interference often or every night, which can lead to depression, anxiety, social, and work-related problems. METHODS This randomized, double-blind, placebo-controlled Phase 2b core study (NCT02477332) included adult patients ≥18 years with moderate to severe CSU inadequately controlled with H1 -antihistamines. The current analysis includes patients randomized to receive ligelizumab 72 or 240 mg, omalizumab 300 mg or placebo every 4 weeks (q4w) for five injections over 20 weeks with treatment-free follow-up for 24 weeks. Patients could enter the open-label extension study (NCT02649218) from Week 32 onwards if their weekly urticaria activity score was ≥12, which included an open-label treatment (52 weeks of ligelizumab 240 mg q4w) and a 48-week post-treatment follow-up. Weekly Sleep Interference Scores (SIS7, range 0 [no interference]-21 [substantial interference]), Weekly Activity Interference Score (AIS7), Dermatology Life Quality Index (DLQI) scores, and Overall Work Impairment were assessed. RESULTS Mean baseline SIS7 scores were balanced between the treatment arms for ligelizumab 72 mg (n = 84) and 240 mg (n = 85), omalizumab 300 mg (n = 85), and placebo (n = 43). By Week 12, patients experienced large improvements in sleep interference, with least square mean (standard error) changes from baseline (CFB) in SIS7 of -7.84 (0.58), -7.55 (0.61), -6.98 (0.60), and -5.85 (0.81), respectively. By Week 12, CFB in AIS7 were -8.25 (0.57), -8.25 (0.59), -7.30 (0.60), and -5.62 (0.79), DLQI scores were -9.79 (0.77), -9.93 (0.81), -8.35 (0.79), and -6.99 (1.11), and Overall Work Impairment scores were -28.96 (3.73), -30.76 (3.71), -25.74 (3.91), and -20.13 (5.10) for ligelizumab 72 and 240 mg, omalizumab 300 mg and placebo, respectively. Improvements in each patient-reported outcome were sustained with ligelizumab 240 mg treatment during the extension study. CONCLUSIONS Ligelizumab showed effective and sustained responses in managing sleep interference in patients with CSU, and numerically higher responses than with omalizumab and placebo. Treating the symptoms of CSU with ligelizumab improved disease burden, HRQoL, and markedly improved sleep quality.
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Affiliation(s)
- Ana Giménez‐Arnau
- Department of DermatologyHospital del MarInstitut Mar d´Investigacions MèdiquesUniversitat AutònomaBarcelonaSpain
| | - Marcus Maurer
- Department of Dermatology and AllergyDermatological AllergologyAllergie‐Centrum‐CharitéCharité ‐ Universitätsmedizin BerlinBerlinGermany
| | - Jonathan Bernstein
- Bernstein Allergy Group and Bernstein Clinical Research CenterCollege of MedicineCincinnatiOhioUSA
| | - Petra Staubach
- Department of DermatologyUniversity Medical Center MainzMainzGermany
| | | | - Eva Hua
- China Novartis Institutes for Biomedical Research Co. LtdShanghaiChina
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Maurer M, Giménez‐Arnau A, Bernstein JA, Chu C, Danilycheva I, Hide M, Makris M, Metz M, Savic S, Sitz K, Soong W, Staubach P, Sussman G, Barve A, Burciu A, Hua E, Janocha R, Severin T. Sustained safety and efficacy of ligelizumab in patients with chronic spontaneous urticaria: A one-year extension study. Allergy 2021; 77:2175-2184. [PMID: 34773261 DOI: 10.1111/all.15175] [Citation(s) in RCA: 28] [Impact Index Per Article: 7.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/27/2021] [Revised: 10/08/2021] [Accepted: 10/18/2021] [Indexed: 12/12/2022]
Abstract
BACKGROUND Ligelizumab, a next-generation, humanized anti-immunoglobulin E (IgE) monoclonal antibody is in development as a treatment for patients with chronic spontaneous urticaria, whose symptoms are inadequately controlled with standard-of-care therapy. OBJECTIVE To evaluate the long-term safety and re-treatment efficacy of ligelizumab 240 mg in patients who completed the core study and extension study. METHODS This open-label, single-arm, long-term Phase 2b extension study was designed to assess patients who were previously administered various doses of ligelizumab, omalizumab or placebo in the Phase 2b, dose-finding core study and who presented with active disease after Week 32. In the extension study, patients received ligelizumab 240 mg subcutaneously every 4 weeks, for 52 weeks and were monitored post-treatment for 48 weeks. RESULTS Overall, ligelizumab was well-tolerated with no newly identified safety signals. A total of 95.4% (226/237) screened patients received ligelizumab 240 mg in the extension study; 84.1% (190/226) of patients experienced at least one treatment-emergent adverse event. Most reported events were mild (41.6%) or moderate (35.8%) and mostly unrelated to the study treatment. At Week 12, 46.5% of patients had a complete response increasing to 53.1% after 52 weeks. Following 52 weeks of extension study treatment, 75.8% (95% confidence interval, 69.9, 81.3) of patients had cumulative complete responses. The median time to relapse in complete responders was 38 weeks. CONCLUSION The long-term safety profile of ligelizumab 240 mg in patients with chronic spontaneous urticaria was consistent with the core study and re-treatment efficacy was shown. TRIAL REGISTRATION ClinicalTrials.gov Identifier: NCT02477332 and NCT02649218.
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Affiliation(s)
- Marcus Maurer
- Dermatological Allergology Allergie‐Centrum‐Charité Department of Dermatology and Allergy Charité – Universitätsmedizin Berlin Berlin Germany
| | - Ana Giménez‐Arnau
- Dermatology Department, Hospital del Mar IMIM Universitat Autònoma Barcelona Barcelona Spain
| | - Jonathan A. Bernstein
- University of Cincinnati College of Medicine and Bernstein Clinical Research Center Cincinnati Ohio USA
| | - Chia‐Yu Chu
- Department of Dermatology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan
| | - Inna Danilycheva
- National Research Center – Institute of Immunology Federal Medical‐Biological Agency of Russia Moscow Russia
| | - Michihiro Hide
- Department of Dermatology Hiroshima University Hiroshima Japan
| | - Michael Makris
- Allergy Unit 2nd Department of Dermatology and Venereology National and Kapodistrian University"Attikon” University Hospital Athens Greece
| | - Martin Metz
- Dermatological Allergology Allergie‐Centrum‐Charité Department of Dermatology and Allergy Charité – Universitätsmedizin Berlin Berlin Germany
| | - Sinisa Savic
- Leeds Biomedical Research Centre Department of Clinical Immunology and Allergy Leeds Institute of Rheumatic and Musculoskeletal Medicine (LIRMM)St James's University Hospital Leeds UK
| | - Karl Sitz
- Little Rock Allergy and Asthma Clinic Little Rock Arkansas USA
| | - Weily Soong
- Alabama Allergy & Asthma Center – AllerVie Health Clinical Research Center of Alabama Birmingham Alabama USA
| | - Petra Staubach
- Department of Dermatology University Medical Center Mainz Germany
| | - Gordon Sussman
- Division of Allergy and Clinical Immunology University of Toronto Canada
| | - Avantika Barve
- Novartis Pharmaceuticals Corporation East Hanover New Jersey USA
| | | | - Eva Hua
- China Novartis Institutes for Biomedical Research Co. Ltd Shanghai China
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Kocatürk E, Al‐Ahmad M, Krause K, Gimenez‐Arnau AM, Thomsen SF, Conlon N, Marsland A, Savk E, Criado RF, Danilycheva I, Fomina D, Godse K, Khoshkhui M, Gelincik A, Degirmentepe EN, Demir S, Ensina LF, Kasperska‐Zajac A, Rudenko M, Valle S, Medina I, Bauer A, Zhao Z, Staubach P, Bouillet L, Küçük ÖS, Ateş C, Maurer M. Effects of pregnancy on chronic urticaria: Results of the PREG-CU UCARE study. Allergy 2021; 76:3133-3144. [PMID: 34022061 DOI: 10.1111/all.14950] [Citation(s) in RCA: 15] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/23/2021] [Revised: 04/23/2021] [Accepted: 05/01/2021] [Indexed: 12/18/2022]
Abstract
BACKGROUND Chronic urticaria (CU) predominantly affects women, and sex hormones can modulate disease activity in female CU patients. As of now, the impact of pregnancy on CU is largely unknown. AIM To analyze the course and features of CU during and after pregnancy. PATIENTS AND METHODS PREG-CU is an international, multicenter study of the Urticaria Centers of Reference and Excellence (UCARE) network. Data were collected via a 47-item questionnaire completed by CU patients, who became pregnant within the last 3 years. RESULTS A total of 288 pregnancies of 288 CU patients from 13 countries were analyzed (mean age at pregnancy: 32.1 ± 6.1 years, duration of CU: 84.9 ± 74.5 months; CSU 66.9%, CSU + CIndU 20.3%, CIndU 12.8%).During pregnancy, 51.1% of patients rated their CU as improved, 28.9% as worse, and 20.0% as unchanged.CU exacerbations most commonly occurred exclusively during the third trimester (in 34 of 124 patients; 27.6%) or the first (28 of 124; 22.8%). The risk factors for worsening of CU during pregnancy were having mild disease and no angioedema before pregnancy, not taking treatment before pregnancy, CIndU, CU worsening during a previous pregnancy, treatment during pregnancy, and stress as a driver of exacerbations. After giving birth, urticaria disease activity remained unchanged in 43.8% of CU patients, whereas 37.4% and 18.1% experienced worsening and improvement, respectively. CONCLUSIONS These results demonstrate the complex impact of pregnancy on the course of CU and help to better counsel patients who want to become pregnant and to manage CU during pregnancy.
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Affiliation(s)
- Emek Kocatürk
- Department of Dermatology Urticaria Center of Reference and Excellence (UCARE) Koç University School of Medicine Istanbul Turkey
| | - Mona Al‐Ahmad
- Microbiology Department Faculty of Medicine Urticaria Center of Reference and Excellence (UCARE) Kuwait University Safat Kuwait
| | - Karoline Krause
- Department of Dermatology and Allergy Urticaria Center of Reference and Excellence (UCARE) Charité ‐ Universitätsmedizin Berlin Berlin Germany
| | - Ana M. Gimenez‐Arnau
- Department of Dermatology Urticaria Center of Reference and Excellence (UCARE) Hospital del Mar IMIM Universitat Autònoma Barcelona Spain
| | - Simon Francis Thomsen
- Department of Dermatology Urticaria Center of Reference and Excellence (UCARE) Bispebjerg Hospital Copenhagen Denmark
| | - Niall Conlon
- Dermatology, and Immunology Urticaria Center of Reference and Excellence (UCARE) St James's Hospital Dublin Ireland
| | - Alexander Marsland
- Department of Dermatology Urticaria Center of Reference and Excellence (UCARE) The Urticaria Clinic Salford Royal Foundation Trust University of Manchester Manchester UK
| | - Ekin Savk
- Aydın Adnan Menderes University Aydın Turkey
| | - Roberta F. Criado
- Urticaria Center of Reference and Excellence (UCARE) Faculdade de Medicina do ABC (FMABC Santo André Brazil
| | | | - Daria Fomina
- First Moscow State Medical University Moscow Russia
- Urticaria Center of Reference and Excellence (UCARE) Moscow Center of Allergy and Immunology Clinical Hospital 52 Ministry of Moscow Healthcare Moscow Russia
| | - Kiran Godse
- Dr. D.Y. Patil Medical College & Hospital Mumbai India
| | - Maryam Khoshkhui
- Allergy Research Center Mashhad University of Medical Sciences Mashhad Iran
| | - Aslı Gelincik
- Istanbul Faculty of Medicine Istanbul University Istanbul Turkey
| | | | - Semra Demir
- Istanbul Faculty of Medicine Istanbul University Istanbul Turkey
| | | | - Alicja Kasperska‐Zajac
- European Center for Diagnosis and Treatment of Urticaria (GA2LEN UCARE Network) Medical University of Silesia in Katowice Katowice Poland
| | | | - Solange Valle
- Federal University of Rio de Janeiro Rio De Janeiro Brazil
| | - Iris Medina
- The Centro Médico Vitae Buenos Aires Argentina
| | - Andrea Bauer
- Department of Dermatology University Allergy Center University Hospital Carl Gustav Carus Technical University Dresden Dresden Germany
| | - Zuotao Zhao
- Department of Dermatology and Venerology Beijing Key Laboratory of Molecular Diagnosis on Dermatoses and National Clinical Research Center for Skin and Immune Diseases Peking University First Hospital Beijing China
| | | | | | | | - Can Ateş
- Department of Biostatistics Aksaray University School of Medicine Aksaray Turkey
| | - Marcus Maurer
- Dermatological Allergology Allergie‐Centrum‐Charité Department of Dermatology and Allergy Urticaria Center of Reference and Excellence (UCARE) Charité – Universitätsmedizin Berlin Berlin Germany
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A validation study of the Japanese version of the Angioedema Activity Score (AAS) and the Angioedema Quality of Life Questionnaire (AE-QoL). Allergol Int 2021; 70:471-479. [PMID: 34023225 DOI: 10.1016/j.alit.2021.04.006] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/20/2020] [Revised: 03/23/2021] [Accepted: 04/16/2021] [Indexed: 12/31/2022] Open
Abstract
BACKGROUND Recurrent angioedema (RecAE) has a substantial impact on patients' daily lives. However, there have been no disease-specific patient-reported outcomes (PROs) available in Japan to measure disease activity and health-related QoL impairment in such patients. METHODS Japanese versions of the Angioedema Activity Score (AAS) and the Angioedema Quality of Life Questionnaire (AE-QoL) were examined for their validity and reliability. By using these questionnaires, the relationship between disease activity and QoL impairment among the Japanese population of RecAE were analyzed in real-world setting. RESULTS The Japanese AAS and AE-QoL domains showed good internal consistency of 0.967 and > 0.835. For known group validity, AAS28 and AE-QoL total scores were higher in more severe patients than those with milder disease and QoL impairment, respectively. AAS28 showed strong correlation with indexes of disease activity, while the AE-QoL total score correlated with Dermatology Life Quality Index (DLQI). Sufficient reproductivity of the AAS and AE-QoL was shown by their intraclass correlation coefficients of 0.890 and 0.700. The Japanese population is characterized by the total score of AAS28, 34.3 ± 38.8 (mean ± SD); and AE-QoL, 38.7 ± 25.2. Each domain score of AE-QoL was 32.4 ± 29.7 in "Functioning", 35.0 ± 27.8 in "Fatigue/mood", 50.7 ± 30.6 in "Fears/shame", or 24.7 ± 29.8 in "Food". Changes in AAS28 and AE-QoL positively correlated to Patient global assessment of disease activity and DLQI, respectively. CONCLUSIONS The Japanese AAS and AE-QoL are valid and reliable instruments for Japanese patients with RecAE, and active disease affecting QoL. They help assess disease activity and QoL of RecAE in routine patient care and clinical trials.
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Maurer M, Magerl M. Differences and Similarities in the Mechanisms and Clinical Expression of Bradykinin-Mediated vs. Mast Cell-Mediated Angioedema. Clin Rev Allergy Immunol 2021; 61:40-49. [PMID: 33534062 PMCID: PMC8282544 DOI: 10.1007/s12016-021-08841-w] [Citation(s) in RCA: 35] [Impact Index Per Article: 8.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/13/2021] [Indexed: 12/22/2022]
Abstract
Angioedema (AE), transient localized swelling due to extravasated fluid, is commonly classified as mast cell mediator-induced, bradykinin-mediated or of unknown cause. AE often occurs more than once, and it is these recurrent forms of AE that are challenging for patients and physicians, and they are the ones we focus on and refer to as AE in this review. Since effective treatment depends on the causative mediator, reliable and early diagnosis is essential. Although their clinical presentations bear similarities, many forms of angioedema exhibit specific patterns of clinical appearance or disease history that may aid in diagnosis. Here, we describe the most common differences and similarities in the mechanisms and clinical features of bradykinin-mediated and mast cell mediator-induced types of angioedema. We first provide an overview of the diseases that manifest with mast cell mediator-induced versus bradykinin-mediated angioedema as well as their respective underlying pathogenesis. We then compare these diseases for key clinical features, including angioedema location, course and duration of swelling, attack frequency, prevalence and relevance of prodromal signs and symptoms, triggers of angioedema attacks, and other signs and symptoms including wheals, age of onset, and duration. Our review and comparison of the clinical profiles of different types of angioedema incorporate our own clinical experience as well as published information. Our aim is to highlight that mast cell mediator-induced and bradykinin-mediated angioedema types share common features but are different in many aspects. Knowledge of the differences in underlying pathomechanisms and clinical profiles between different types of angioedema can help with the diagnostic approach in affected patients and facilitate targeted and effective treatment.
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Affiliation(s)
- Marcus Maurer
- Dermatological Allergology, Allergie-Centrum-Charité, Department of Dermatology and Allergy, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany.
| | - Markus Magerl
- Dermatological Allergology, Allergie-Centrum-Charité, Department of Dermatology and Allergy, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany
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Can PK, Degi Rmentepe EN, Etikan P, Kiziltaç K, Gelincik A, Demir S, Buyukozturk S, Haşal E, Bülbül Başkan E, Aydin Ö, Maurer M, Weller K, Kocaturk E. Assessment of disease activity and quality of life in patients with recurrent bradykinin-mediated versus mast cell-mediated angioedema. World Allergy Organ J 2021; 14:100554. [PMID: 34221217 PMCID: PMC8219995 DOI: 10.1016/j.waojou.2021.100554] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/02/2021] [Revised: 04/30/2021] [Accepted: 05/21/2021] [Indexed: 11/08/2022] Open
Abstract
Objective Recurrent Angioedema (RAE) is characterized by sudden swelling of mucosal surfaces or deep dermis and is either mast cell-(MMAE) or bradykinin-mediated (BMAE). How patients with BMAE and MMAE differ in terms of disease activity and impact remains largely unknown. Here, we determined validity, reliability, and sensitivity to change of Turkish versions of angioedema activity score (AAS) and quality of life questionnaire (AE-QoL) and used both instruments to investigate and compare patients with BMAE and MMAE. Methods Turkish versions of AAS28 and AE-QoL were applied to 94 patients with RAE (18–72 years). Patients’ global self-assessment of QoL (PGA-QoL), disease activity (PGA-DA-VRS, PatGA-DA-VAS), and 12-Item-Short Form Survey were used at week 4 (visit 2), and week 8 (visit 3). Demographic characteristics, clinical features, and AAS28 and AE-QoL values were compared between 31 patients with BMAE and 63 patients with MMAE. Results Turkish AAS28 and AE-QoL showed excellent internal consistency, high reproducibility and known-groups validity. Compared to patients with MMAE, BMAE patients were younger (34.6 ± 10.7 vs. 40.7 ± 13.3 years), had longer disease duration (236 ± 178 vs. 51 ± 78 months), high prevalence of family history (63% vs 14%), longer duration of attacks (65 ± 20 vs. 40 ± 25 h), and they were more commonly affected by upper airway angioedema (70% vs 23%). Disease activity (AAS28) was lower (29.3 ± 24.6 vs 55.2 ± 52.9), but AE-QoL was higher (44.2 ± 16.1 vs 34.5 ± 22.5) in BMAE patients as compared to MMAE patients. Conclusions Patients with BMAE and MMAE have distinct disease characteristics. Recurrent bradykinin-mediated angioedema impacts quality of life more than mast cell-mediated angioedema. The discriminating characteristics of patients with BMAE and MMAE may help to improve the diagnosis and management of patients with RAE.
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Affiliation(s)
- Pelin Kuteyla Can
- Bahcesehir University, Faculty of Medicine, Department of Dermatology, Istanbul, Turkey
| | - Ece Nur Degi Rmentepe
- Okmeydani Training and Research Hospital, Dermatology and Venerology, Istanbul, Turkey
| | - Piril Etikan
- Okmeydani Training and Research Hospital, Dermatology and Venerology, Istanbul, Turkey
| | - Kübra Kiziltaç
- Okmeydani Training and Research Hospital, Dermatology and Venerology, Istanbul, Turkey
| | - Asli Gelincik
- Istanbul University, Istanbul Faculty of Medicine, Adult Allergy Clinic, Istanbul, Turkey
| | - Semra Demir
- Istanbul University, Istanbul Faculty of Medicine, Adult Allergy Clinic, Istanbul, Turkey
| | - Suna Buyukozturk
- Istanbul University, Istanbul Faculty of Medicine, Adult Allergy Clinic, Istanbul, Turkey
| | - Eda Haşal
- Uludağ University Faculty of Medicine Department of Dermatology, Bursa, Turkey
| | - Emel Bülbül Başkan
- Uludağ University Faculty of Medicine Department of Dermatology, Bursa, Turkey
| | - Ömür Aydin
- Ankara University Faculty of Medicine, Adult Allergy Clinic, Ankara, Turkey
| | - Marcus Maurer
- Dermatological Allergology, Allergie-Centrum-Charité, Department of Dermatology and Allergy, Charité - Universitätsmedizin Berlin, Berlin, Germany
| | - Karsten Weller
- Dermatological Allergology, Allergie-Centrum-Charité, Department of Dermatology and Allergy, Charité - Universitätsmedizin Berlin, Berlin, Germany
| | - Emek Kocaturk
- Koç University School of Medicine Department of Dermatology, Istanbul, Turkey
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Sánchez-Borges M, Ansotegui IJ, Baiardini I, Bernstein J, Canonica GW, Ebisawa M, Gomez M, Gonzalez-Diaz SN, Martin B, Morais-Almeida M, Ortega Martell JA. The challenges of chronic urticaria part 1: Epidemiology, immunopathogenesis, comorbidities, quality of life, and management. World Allergy Organ J 2021; 14:100533. [PMID: 34221215 PMCID: PMC8233382 DOI: 10.1016/j.waojou.2021.100533] [Citation(s) in RCA: 9] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/07/2021] [Revised: 02/18/2021] [Accepted: 03/08/2021] [Indexed: 12/02/2022] Open
Abstract
This is Part 1 of an updated follow-up review of a World Allergy Organization (WAO) position paper published in 2012 on the diagnosis and treatment of urticaria and angioedema. Since 2012, there have been advances in the understanding of the pathogenesis of chronic urticaria, and greater experience with the use of biologics, such as omalizumab, in patients with severe refractory disease. For these reasons, the WAO decided to initiate an update targeted to general practitioners around the world, incorporating the most recent information on epidemiology, immunopathogenesis, comorbidities, quality of life, clinical case presentations, and the management of chronic spontaneous and chronic inducible urticaria, including urticaria in special situations such as childhood and pregnancy. A special task force of WAO experts was invited to write the different sections of the manuscript, and the final document was approved by the WAO Board of Directors. This paper is not intended to be a substitute for current national and international guidelines on the management of urticaria and angioedema but to provide an updated, simplified guidance for physicians around the world who manage patients with this common ailment.
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Affiliation(s)
- Mario Sánchez-Borges
- Allergy and Clinical Immunology Department, Centro Médico Docente La Trinidad, and Clínica El Avila, Caracas, Venezuela
| | - Ignacio J. Ansotegui
- Department of Allergy and Immunology, Hospital Quirónsalud Bizkaia, Bilbao, Spain
| | - Ilaria Baiardini
- Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Personalized Medicine, Asthma and Allergy, Humanitas Clinical and Research Center IRCCS, Rozzano, Italy
| | - Jonathan Bernstein
- Department of Internal Medicine, Division of Immunology, Allergy Section, University of Cincinnati
| | - Giorgio Walter Canonica
- Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Personalized Medicine, Asthma and Allergy, Humanitas Clinical and Research Center IRCCS, Rozzano, Italy
| | - Motohiro Ebisawa
- Clinical Research Center for Allergy and Rheumatology, National Hospital Organization, Sagamihara National Hospital, Sagamihara, Kanagawa, Japan
| | | | - Sandra Nora Gonzalez-Diaz
- Regional Center for Allergy and Clinical Immunology, Faculty of Medicine and “Dr. José Eleuterio González" University Hospital, Autonomous University of Nuevo León, Monterrey, México
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Moos Ł, Kapeluszna K, Okuniewicz R, Brzoza Z. The Role of Interleukin 10 and 18 in Chronic Spontaneous Urticaria Pathogenesis in the Context of Angioedema Coexistence. J Interferon Cytokine Res 2021; 41:172-176. [PMID: 34003678 DOI: 10.1089/jir.2020.0256] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/03/2023] Open
Abstract
Chronic spontaneous urticaria (CSU) is defined using clinical symptoms as spontaneous occurrence of itchy wheals and/or angioedema for at least 6 weeks. Angioedema is underdiagnosed in CSU patients, and its presence has significant negative impact on health-related quality of life, daily activities, health care resource utilization, and work. Various cytokines have been found to be involved in pathogenesis of CSU. To study levels of interleukin (IL)-10 and IL-18 in CSU patients and to look for the differences in CSU subgroups divided with regard to angioedema reoccurrence, we included consecutive CSU patients into the study. To assess disease activity, urticaria activity score was used. In addition, we calculated disease duration time. In all groups, Il-10 and Il-18 serum concentrations were measured. The study involved 52 patients with CSU and 47 healthy volunteers. The IL-10 level was statistically significantly higher in patients with CSU compared to the control group. There were no significant differences in level of IL-18 between those groups. Comparison of patients with CSU and angioedema with those without angioedema showed no significant differences in level of IL-10 and IL-18. We see the need for further studies of serum levels of IL-10 and IL-18 to better understand the pathogenesis of the disease and to find markers useful in predicting the symptom type in the course of CSU.
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Affiliation(s)
- Łukasz Moos
- Department of Internal Medicine with Division of Allergology, Institute of Medical Sciences, University of Opole, Opole, Poland
| | - Katarzyna Kapeluszna
- Department of Internal Medicine with Division of Allergology, Institute of Medical Sciences, University of Opole, Opole, Poland
| | - Robert Okuniewicz
- Department of Internal Medicine with Division of Allergology, Institute of Medical Sciences, University of Opole, Opole, Poland
| | - Zenon Brzoza
- Department of Internal Medicine with Division of Allergology, Institute of Medical Sciences, University of Opole, Opole, Poland
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Sabaté-Brescó M, Rodriguez-Garijo N, Azofra J, Baeza ML, Donado CD, Gaig P, Guilarte M, Herrera-Lasso V, Labrador-Horrillo M, Sala-Cunill A, Veleiro B, Gil MP, Kaplan A, Ferrer M. A Comparative Study of Sex Distribution, Autoimmunity, Blood, and Inflammatory Parameters in Chronic Spontaneous Urticaria with Angioedema and Chronic Histaminergic Angioedema. THE JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE 2021; 9:2284-2292. [PMID: 33831619 DOI: 10.1016/j.jaip.2021.03.038] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 01/01/2021] [Revised: 03/15/2021] [Accepted: 03/18/2021] [Indexed: 10/21/2022]
Abstract
BACKGROUND Recurrent idiopathic histaminergic angioedema is currently classified as a subtype of angioedema, as well as a subtype of chronic spontaneous urticaria (CSU), based on the fact that both are mast cell-mediated and respond to the same treatments. OBJECTIVE In the present work, we sought to verify whether chronic histaminergic angioedema (CHA) is an entity distinct from CSU or represents a CSU subtype that lacks hives. METHODS We performed a prospective study comparing 68 CHA patients, angioedema without hives, with 63 CSU patients, with hives and angioedema, from whom we collected demographic and clinical data, as well as blood and serum markers. RESULTS We found key pathogenic features that differentiate CHA from CSU: gender distribution, basophil number, and antibodies against the IgE receptor. The male/female ratio in CHA was 0.78, whereas in CSU it was 0.36 (P = .0466). Basopenia was more often seen in CSU (n = 13 [20%]) than in CHA (n = 5 [7%]). Finally, 31.15% of CSU sera induced basophil activation, whereas no CHA sera were able to activate normal basophils. By contrast, nonspecific inflammation or immune markers, for example, erythrocyte sedimentation rate, C-reactive protein, or IgG antithyroid antibodies, were very similar between both groups. IgE anti-IL-24 could not be assessed because a control population did not differ from CSU. CONCLUSIONS Inclusion of CHA as part of the spectrum of CSU is an assumption not evidence-based, and when studied separately, important differences were observed. Until there is further evidence, CHA and CSU should not necessarily be considered the same disorder, and it is our opinion that review articles and guidelines should reflect that possibility.
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Affiliation(s)
- Marina Sabaté-Brescó
- Department of Allergy and Clinical Immunology, Clínica Universidad de Navarra, Pamplona, Spain; Instituto de Investigación Sanitaria de Navarra (IdiSNA), Pamplona, Spain; RETIC de Asma, Reacciones Adversas y Alérgicas (ARADYAL), Madrid, Spain
| | | | - Julian Azofra
- Allergy Section, Hospital Universitario Central de Asturias, Oviedo, Spain
| | - Maria Luisa Baeza
- Allergy Service, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Biomedical Research Network on Rare Diseases (CIBERER)-U761, Madrid, Spain; Gregorio Marañón Health Research Institute, Madrid, Spain
| | - Carmen D Donado
- Allergy Section, Hospital Universitario Central de Asturias, Oviedo, Spain
| | - Pere Gaig
- Allergy Section, Hospital Universitari Joan XXIII de Tarragona, Tarragona, Spain; Departament de Medicina i Cirurgia, Institut d'Investigació Sanitària Pere Virgili, Universitat Rovira i Virgili, Tarragona, Spain
| | - Mar Guilarte
- RETIC de Asma, Reacciones Adversas y Alérgicas (ARADYAL), Madrid, Spain; Allergy Section, Internal Medicine Department, Hospital Universitari Vall d'Hebron, Barcelona, Spain
| | - Valeria Herrera-Lasso
- Allergy Section, Hospital Universitari Joan XXIII de Tarragona, Tarragona, Spain; Departament de Medicina i Cirurgia, Institut d'Investigació Sanitària Pere Virgili, Universitat Rovira i Virgili, Tarragona, Spain
| | - Moisés Labrador-Horrillo
- RETIC de Asma, Reacciones Adversas y Alérgicas (ARADYAL), Madrid, Spain; Allergy Section, Internal Medicine Department, Hospital Universitari Vall d'Hebron, Barcelona, Spain
| | - Anna Sala-Cunill
- RETIC de Asma, Reacciones Adversas y Alérgicas (ARADYAL), Madrid, Spain; Allergy Section, Internal Medicine Department, Hospital Universitari Vall d'Hebron, Barcelona, Spain
| | - Beatriz Veleiro
- Department of Allergy and Clinical Immunology, Complexo Hospitalario Universitario de A Coruña, A Coruña, Spain
| | - María Pilar Gil
- Department of Dermatology, Clinica Universidad de Navarra, Pamplona, Spain
| | - Allen Kaplan
- Department of Medicine, The Medical University of South Carolina, Charleston, SC
| | - Marta Ferrer
- Department of Allergy and Clinical Immunology, Clínica Universidad de Navarra, Pamplona, Spain; Instituto de Investigación Sanitaria de Navarra (IdiSNA), Pamplona, Spain; RETIC de Asma, Reacciones Adversas y Alérgicas (ARADYAL), Madrid, Spain.
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Bauer A, Dickel H, Jakob T, Kleinheinz A, Lippert U, Metz M, Schliemann S, Schwichtenberg U, Staubach P, Valesky E, Wagner N, Wedi B, Maurer M. Expertenkonsensus zu praxisrelevanten Aspekten bei der Behandlung der chronischen Urtikaria. ALLERGO JOURNAL 2021; 30:40-55. [PMID: 33785991 PMCID: PMC7994058 DOI: 10.1007/s15007-021-4772-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
Hintergrund: Die chronische Urtikaria (CU) ist eine häufige und für viele Patienten stark belastende Erkrankung. Die aktuelle Urtikaria-Leitlinie beschreibt die evidenzbasierte Diagnostik und Therapie der CU. Darüber hinaus treten jedoch oftmals Fragen im praktischen Alltag auf, die durch die Leitlinie nicht beantwortet werden. Methoden: Im Mai 2020 fand ein digitales Treffen deutscher Urtikaria-Experten statt, in dem praxisrelevante Aspekte der Behandlung der CU erörtert und unterstützende Hilfestellungen für den klinischen Behandlungsalltag formuliert wurden. Die resultierenden Hinweise dieses Dokumentes fokussieren auf praktische Fragen und der hierzu verfügbaren Literatur und Erfahrungen der Teilnehmer. Ergebnisse: Die Diagnose einer CU kann in kurzer Zeit mittels gründlicher Anamnese, einer körperlichen Untersuchung und einer laborchemischen Basisdiagnostik gestellt werden. Hierzu werden in dieser Arbeit praktische Empfehlungen für den Praxisalltag gegeben. Eine erweiterte Diagnostik ist nur in wenigen Fällen indiziert und sollte grundsätzlich parallel zu einer effektiven Therapie erfolgen. Generell gilt, dass die CU immer auf gleiche Weise zu therapieren ist, unabhängig davon, ob Quaddeln, Angioödeme oder beides auftreten. Eine symptomatische Therapie sollte nach dem von den Leitlinien empfohlenen Stufenschema erfolgen. Die vorliegende Publikation gibt hierzu praktische Hinweise für Fragen in der Praxis, wie zum Beispiel dem Vorgehen in der aktuellen COVID-19-Pandemie, dem kardialen Risiko unter höher dosierten H 1-Antihistaminika, der Selbstapplikation von Omalizumab sowie dem Impfen unter Omalizumab-Therapie. Zusätzlich zu den Behandlungsempfehlungen werden Themen wie die Dokumentation in der Praxis und Familienplanung bei Urtikaria besprochen. Diskussion: Diese unterstützenden Behandlungsempfehlungen dienen als Ergänzung zu den aktuellen Leitlinien der CU und geben beim Umgang mit Patienten mit CU eine Hilfestellung für den Praxisalltag. Ziel ist es, dass Patienten, die unter einer CU leiden, mithilfe einer optimalen Therapie eine vollständige Beschwerdefreiheit erreichen.
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Affiliation(s)
- Andrea Bauer
- Universitätsklinikum Carl Gustav Carus \/ TU Dresden, Fetscherstr. 74, 1307 Dresden, Germany
| | - Heinrich Dickel
- Klinik für Dermatologie, Venerologie und Allergologie, Gudrunstr. 56, 44791 Bochum, Germany
| | - Thilo Jakob
- Universitäts-Hautklinik Gießen, Gießen, Germany
| | | | - Undine Lippert
- Universitätsmedizin Göttingen, Klinik für Dermatologie, Venerologie und Allergologie, Göttingen, Germany
| | - Martin Metz
- Charité – Universitätsmedizin Berlin, Klinik für Dermatologie, Venerologie und Allergolo, Berlin, Germany
| | | | | | | | - Eva Valesky
- Universitätsklinikum Frankfurt, Klinik für Dermatologie, Venerologie und Allergologie, Frankfurt, Germany
| | - Nicola Wagner
- Klinikum Darmstadt, Heidelberger Landstraße 379, 64297 Darmstadt-Eberstadt, Germany
| | - Bettina Wedi
- Universitätsmedizin Göttingen, Klinik für Dermatologie, Venerologie und Allergologie, Göttingen, Germany
| | - Marcus Maurer
- Charité-Universitätsmedizin Berlin, Charitéplatz 1, 10117 Berlin, Germany
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Bauer A, Dickel H, Jakob T, Kleinheinz A, Lippert U, Metz M, Schliemann S, Schwichtenberg U, Staubach P, Valesky E, Wagner N, Wedi B, Maurer M. Expert consensus on practical aspects in the treatment of chronic urticaria. ACTA ACUST UNITED AC 2021; 30:64-75. [PMID: 33643777 PMCID: PMC7903036 DOI: 10.1007/s40629-021-00162-w] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/18/2020] [Accepted: 12/30/2020] [Indexed: 12/13/2022]
Abstract
Background Chronic urticaria (CU) is a common disease which represents a considerable burden for many patients. The current urticaria guideline describes the evidence-based diagnosis and treatment of CU. In addition, however, questions often arise in everyday practice that are not addressed by the guideline. Methods In May 2020, a digital meeting with German urticaria experts was held, in which practical aspects of CU treatment were discussed and supporting aids for everyday clinical treatment formulated. The resulting advice in this document focus on practical questions and the available literature and experiences of the participants. Results The diagnosis of CU can be made in a short time by means of a thorough anamnesis, a physical examination, and a basic laboratory chemical diagnosis. For this purpose, practical recommendations for everyday practice are given in this paper. An extended diagnosis is only indicated in a few cases and should always be carried out in parallel with an effective therapy. In general, CU should always be treated in the same way, regardless of whether wheals, angioedema or both occur. Symptomatic therapy should be carried out according to the treatment steps recommended by the guidelines. This publication provides practical advice on issues in everyday practice, such as the procedure in the current coronavirus disease 2019 (COVID-19) pandemic, the cardiac risk under higher dosed H1 antihistamines, the self-administration of omalizumab as well as vaccination under omalizumab therapy. In addition to treatment recommendations, topics such as documentation in the practice and family planning with urticaria will be discussed. Discussion These supporting treatment recommendations serve as an addendum to the current CU guideline and provide support in dealing with CU patients in everyday practice. The aim is to ensure that patients suffering from CU achieve complete freedom of symptoms with the help of an optimal therapy. Supplementary Information The online version of this article (10.1007/s40629-021-00162-w) contains supplementary material, which is available to authorized users.
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Affiliation(s)
- Andrea Bauer
- University Hospital Carl Gustav Carus, Department of Dermatology, University Allergy Center, Urticaria Center of Reference and Excellence (UCARE), Technical University, Dresden, Germany
| | - Heinrich Dickel
- Department of Dermatology, Venereology and Allergology, St. Josef Hospital, Ruhr University Bochum, Bochum, Germany
| | - Thilo Jakob
- University Medical Center Giessen (UKGM), Department of Dermatology and Allergy, Justus-Liebig-University Giessen, Giessen, Germany
| | - Andreas Kleinheinz
- Department of Dermatology, Urticaria Center of Reference and Excellence (UCARE), Elbe Medical Centre, Buxtehude, Germany
| | - Undine Lippert
- Department of Dermatology and Allergology, Urticaria Center of Reference and Excellence (UCARE), University Medical Center Göttingen, Göttingen, Germany
| | - Martin Metz
- Department of Dermatology and Allergy, Urticaria Center of Reference and Excellence (UCARE), Charité-Universitätsmedizin Berlin, Charitéplatz 1, 10117 Berlin, Germany
| | - Sibylle Schliemann
- Department of Dermatology, Urticaria Center of Reference and Excellence (UCARE), University Hospital Jena, Jena, Germany
| | | | - Petra Staubach
- Department of Dermatology, Urticaria Center of Reference and Excellence (UCARE), University Medical Center Mainz, Mainz, Germany
| | - Eva Valesky
- University Hospital Frankfurt, Department of Dermatology, Venerology and Allergology, Goethe University, Frankfurt, Germany
| | - Nicola Wagner
- Department of Dermatology, Urticaria Center of Reference and Excellence (UCARE), University Medical Center Erlangen, Erlangen, Germany
| | - Bettina Wedi
- Department of Dermatology and Allergy, Urticaria Center of Reference and Excellence (UCARE), Comprehensive Allergy Center, Hannover Medical School, Hannover, Germany
| | - Marcus Maurer
- Department of Dermatology and Allergy, Urticaria Center of Reference and Excellence (UCARE), Charité-Universitätsmedizin Berlin, Charitéplatz 1, 10117 Berlin, Germany
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