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Taniguchi T, Hiwa R, Shoji M, Yamaguchi E, Shirakashi M, Onizawa H, Tsuji H, Kitagori K, Nakashima R, Akizuki S, Onishi A, Yoshifuji H, Tanaka M, Morinobu A. Avacopan's potential to decrease MPO-ANCA titres concurrent with ameliorated activity in ANCA-associated vasculitis. Mod Rheumatol Case Rep 2024; 8:314-317. [PMID: 38564324 DOI: 10.1093/mrcr/rxae016] [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: 02/22/2024] [Accepted: 03/13/2024] [Indexed: 04/04/2024]
Abstract
Avacopan, an orally administered C5a receptor antagonist, is effective in microscopic polyangiitis via the inhibition of neutrophil priming induced by C5a. However, the exact effect of avacopan on the production of myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) is yet to be clearly established. This report presents a microscopic polyangiitis patient without major organ damage where high levels of MPO-ANCA persisted with high-dose steroid therapy and azathioprine, but the addition of avacopan led to a reduction in MPO-ANCA titres. The present case implies that avacopan-mediated inhibition of C5a may lead to a reduction in MPO-ANCA levels, thereby potentially ameliorating the pathophysiology of ANCA-associated vasculitis. Nevertheless, the impact of avacopan on MPO-ANCA production cannot be asserted solely based on this report; therefore, further examination is necessary through subgroup analysis using data from larger-scale studies.
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Affiliation(s)
- Tomoki Taniguchi
- Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Ryosuke Hiwa
- Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Mikihito Shoji
- Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Eriho Yamaguchi
- Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Mirei Shirakashi
- Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Hideo Onizawa
- Department of Advanced Medicine for Rheumatic Diseases, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Hideaki Tsuji
- Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Koji Kitagori
- Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Ran Nakashima
- Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Shuji Akizuki
- Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Akira Onishi
- Department of Advanced Medicine for Rheumatic Diseases, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Hajime Yoshifuji
- Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Masao Tanaka
- Department of Advanced Medicine for Rheumatic Diseases, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Akio Morinobu
- Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
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Vats V, Patel K, Sharma DD, Almansouri NE, Makkapati NSR, Nimal S, Ramteke P, Mohammed Arifuddin B, Jagarlamudi NS, Narain A, Raut YD. Exploring Cardiovascular Manifestations in Vasculitides: An In-Depth Review. Cureus 2023; 15:e44417. [PMID: 37791229 PMCID: PMC10543473 DOI: 10.7759/cureus.44417] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/30/2023] [Indexed: 10/05/2023] Open
Abstract
Systemic vasculitides encompass a cluster of autoimmune diseases that affect blood vessels, and are characterized by immune-mediated injury to either small- or large-sized blood vessels. Individuals afflicted with systemic vasculitides experience notable morbidity and mortality attributable to cardiovascular manifestations. Noteworthy among these are ischemic heart disease, venous thromboembolism, aortic involvement, valvular irregularities, myocarditis, and pericarditis. This narrative review investigated and evaluated the prevalent cardiovascular disturbances commonly associated with different types of vasculitides. This review also discusses the mechanisms that underlie these manifestations. It also provides a thorough explanation of the many diagnostic techniques essential for detecting the disease at its occult stage. It is essential for healthcare professionals to have knowledge of the cardiovascular complications caused by vasculitides, as this enables them to promptly recognize these symptoms and employ suitable diagnostic techniques early on. By doing so, timely detection can be ensured, which will subsequently aid in initiating appropriate treatment strategies that are vital for decreasing morbidity and mortality in patients with systemic vasculitides.
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Affiliation(s)
- Vaibhav Vats
- Internal Medicine, Smt. Kashibai Navale Medical College and General Hospital, Mumbai, IND
| | - Kriyesha Patel
- Internal Medicine, MP Shah Medical College, Jamnagar, IND
| | | | | | | | - Simran Nimal
- Internal Medicine, Byramjee Jeejeebhoy (BJ) Government Medical College, Pune, IND
| | - Palash Ramteke
- Medical School, NKP Salve Institute of Medical Sciences, Nagpur, IND
| | | | | | - Archit Narain
- Internal Medicine, Lala Lajpat Rai Memorial Medical College, Meerut, IND
| | - Yogesh D Raut
- Miscellaneous, NKP Salve Institute of Medical Sciences, Nagpur, IND
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Orlando PBC, Ines SGC, Eduardo RVG, Fernanda ÁBM. An unusual case of heart failure due to ANCA-negative vasculitis: A case report and focused review of the literature. Clin Case Rep 2023; 11:e7749. [PMID: 37546160 PMCID: PMC10397480 DOI: 10.1002/ccr3.7749] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/13/2023] [Revised: 07/09/2023] [Accepted: 07/22/2023] [Indexed: 08/08/2023] Open
Abstract
Key Clinical Message Heart failure due to ANCA-negative vasculitis, is a rare potentially life-threatening manifestation of ANCA vasculitis. Therefore, physicians must always pay attention to this manifestation for proper disease diagnosis and treatment. Abstract Less than 10% of primary vasculitides cause cardiac dysfunction, with Takayasu's arteritis, polyarteritis nodosa, and eosinophilic granulomatosis with polyangiitis being the most common cases. However, any cardiac tissue can be affected by ANCA vasculitis. We present a case of heart failure with reduced ventricular ejection fraction due to ANCA negative-vasculitis.
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Affiliation(s)
| | - Saldarriaga Giraldo Clara Ines
- Department of Cardiology and Heart FailureUniversidad de Antioquía, Universidad Pontificia Bolivariana, Clínica Cardio VIDMedellínColombia
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Zhang X, Zhao GB, Li LK, Wang WD, Lin HL, Yang N. Myeloperoxidase-antineutrophil cytoplasmic antibody-associated vasculitis with headache and kidney involvement at presentation and with arthralgia at relapse: A case report. World J Clin Cases 2023; 11:5167-5172. [PMID: 37583854 PMCID: PMC10424013 DOI: 10.12998/wjcc.v11.i21.5167] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/10/2023] [Revised: 06/12/2023] [Accepted: 07/06/2023] [Indexed: 07/26/2023] Open
Abstract
BACKGROUND Patients with proteinase 3-antineutrophil cytoplasmic antibody associated vasculitis (AAV) experience different manifestations at the initial onset and relapse. However, such cases of different initial and relapse manifestations have not been reported in myeloperoxidase (MPO)-AAV patients. CASE SUMMARY A 52-year-old woman was admitted to our hospital because of headache. Laboratory findings indicated nephrotic range proteinuria and microscopic hematuria, serum creatinine of 243 μmol/L, anti-MPO antibody titer of > 400 RU/mL, and positive perinuclearantineutrophil cytoplasmic antibody. Renal biopsy showed pauci-immune crescentic glomerulonephritis. The cerebrospinal fluid examination and brain magnetic resonance imaging did not show any abnormality. Therefore, MPO-AAV was diagnosed. Corticosteroids, plasmapheresis, and cyclophosphamide as induction therapy and mycophenolate mofetil (MMF) as maintenance therapy were administered. The patient's headache disappeared; serum creatinine returned to normal; complete remission of microscopic hematuria and proteinuria was observed. Anti-MPO antibody titer reached normal limits after immunosuppressive treatment. Twenty-five months after stopping the immunosuppressive treatment, the patient relapsed with arthralgia, without neurological or renal involvement. The patient's arthralgia improved after treatment with prednisone and MMF. CONCLUSION We have reported a rare case of MPO-AAV who initially presented with headache and kidney involvement. However, relapse presented with only arthralgia, which was completely different from the initial manifestations. This case suggests that AAV relapse should be highly suspected in MPO-AAV patients after remission, when clinical manifestations at relapse are different from those at onset. Prednisone and MMF may provide a good choice for refractory arthralgia during relapse in MPO-AAV patients.
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Affiliation(s)
- Xue Zhang
- Graduate School, Dalian Medical University, Dalian 116011, Liaoning Province, China
- Department of Nephrology, The First Affiliated Hospital of Dalian Medical University, Dalian 116011, Liaoning Province, China
| | - Guang-Ben Zhao
- Department of Nephrology, The First Affiliated Hospital of Dalian Medical University, Dalian 116011, Liaoning Province, China
| | - Long-Kai Li
- Department of Nephrology, The First Affiliated Hospital of Dalian Medical University, Dalian 116011, Liaoning Province, China
| | - Wei-Dong Wang
- Department of Nephrology, The First Affiliated Hospital of Dalian Medical University, Dalian 116011, Liaoning Province, China
| | - Hong-Li Lin
- Department of Nephrology, The First Affiliated Hospital of Dalian Medical University, Dalian 116011, Liaoning Province, China
| | - Ning Yang
- Department of Nephrology, The First Affiliated Hospital of Dalian Medical University, Dalian 116011, Liaoning Province, China
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Primary angiitis of central nervous system – A challenging diagnosis. J Neuroimmunol 2022; 366:577844. [DOI: 10.1016/j.jneuroim.2022.577844] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/31/2021] [Revised: 02/26/2022] [Accepted: 03/06/2022] [Indexed: 12/14/2022]
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Diagnostic and Therapeutic Challenges of Vasculitis. Can J Cardiol 2022; 38:623-633. [DOI: 10.1016/j.cjca.2022.02.007] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/25/2021] [Revised: 02/05/2022] [Accepted: 02/07/2022] [Indexed: 12/17/2022] Open
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Clinical and pathological analysis of 101 cases of ANCA-associated vasculitic kidney injury. Int Urol Nephrol 2021; 53:2099-2106. [PMID: 33666803 DOI: 10.1007/s11255-021-02793-y] [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: 06/08/2020] [Accepted: 02/08/2021] [Indexed: 10/22/2022]
Abstract
OBJECTIVE To analyze the clinical and pathological features of patients with anti-neutrophilic cytoplasmic antibody (ANCA)-associated vasculitic (AASV)-kidney injury (AASVKI). METHODS From January 2015 to December 2018, a total of 101 AASVKI patients treated in the First Hospital of Jilin University were divided into 2 groups (the pANCA-positive group and the cANCA-positive group) for comparison; 63 patients were performed renal biopsy and divided into 3 groups according to pathological results [the non-crescent nephritis group (non-C), the crescent nephritis group (C), and the sclerotic nephritis group (S)] for comparison. RESULTS Compared with the Group pANCA, Group cANCA exhibited higher incidence of eye involvement (P = 0.039) and skin mucosa destruction (P = 0.045), higher serum creatinine (Scr) (P < 0.001), higher 24-h urine protein quantification (P = 0.045), but lower hemoglobin (Hb) (P < 0.001). Compared with Group non-C, Group C had higher Scr (P < 0.001) and urinary red blood cells (P = 0.010), lower estimated glomerular filtration rate (eGFR) (P = 0.003), hemoglobin (HD)-dependence (P = 0.042), and higher mortality rate within 1 year (P = 0.005); compared with Group S, Group C had faster onset of erythrocyte sedimentation rate (ESR) (P = 0.031), higher urinary red blood cells (P = 0.029), and lower incidence of HD-dependence (P = 0.038). CONCLUSIONS AASVKI patients with positive cANCA have poor renal function, among whom the patients with crescent type have poorer renal function than those with non-crescent type, have higher urinary red blood cells while lower HD-dependence than those with sclerotic type.
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Liu S, Guo L, Zhang Z, Li M, Zeng X, Wang L, Liu Y, Zhang F. Cardiac manifestations of eosinophilic granulomatosis with polyangiitis from a single-center cohort in China: clinical features and associated factors. Ther Adv Chronic Dis 2021; 12:2040622320987051. [PMID: 33613936 PMCID: PMC7841702 DOI: 10.1177/2040622320987051] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/16/2020] [Accepted: 12/18/2020] [Indexed: 12/26/2022] Open
Abstract
Background: Cardiac manifestations are common and life-threatening in eosinophilic granulomatosis with polyangiitis (EGPA), which remains poorly studied in China. We aim to investigate its clinical features, associated factors, treatment, and outcomes. Methods: We reviewed the clinical records of 110 EGPA patients and examined the independent factors associated with cardiac manifestations using multivariate logistic regression. Receiver operating characteristic curves determined the cut-off values, and survival was calculated via Kaplan–Meier curves. Results: Cardiac involvement was present in 36.4% (40/110) of EGPA patients, which mainly manifested as pericardial effusion (16.4%, 18/110), myocardial involvement (13.6%, 15/110), and heart failure (8.2%, 9/110). The mean age was 42.1 ± 14.23 years with no female/male predominance. Compared with the cardiac-unaffected group, the cardiac-affected group showed a lower rate of biopsy-proved vasculitis (0% versus 20%, p = 0.002). The eosinophil count [odds ratio (OR) = 1.142, 95% confidence interval (CI) 1.029–1.267] was independently associated with cardiac manifestations in EGPA, with a cut-off value of 3.66 × 109/L [area under the curve (AUC) = 0.692, p = 0.001]. Regarding treatment, the cardiac-affected group displayed a higher ratio of glucocorticoid pulse combined with intravenous cyclophosphamide (CYC-IV) (40% versus 21.4%, p = 0.037), and intravenous immunoglobulin combined with glucocorticoid plus CYC-IV (17.5% versus 4.3%, p = 0.035) than the control group. Outcomes (p = 0.131) and survival (p = 0.1972) were not significantly different between the groups. Conclusion: In this single-center Chinese EGPA cohort, cardiac manifestations are observed in 36.4% of patients, which primarily presents as myocardial involvement, pericardial effusion, and heart failure, independently associated with eosinophil count. Glucocorticoid combined with cyclophosphamide is the treatment cornerstone for EGPA patients with cardiac manifestations.
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Affiliation(s)
- Suying Liu
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, The Ministry of Education Key Laboratory, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, China
| | - Ling Guo
- Department of Rheumatology, Dongying People's Hospital, Dongying, Shandong Province, China
| | - Zhaocui Zhang
- Department of Rheumatology and Clinical Immunology, Gansu Province People's Hospital, Lanzhou, Gansu Province, China
| | - Mengtao Li
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, The Ministry of Education Key Laboratory, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, China
| | - Xiaofeng Zeng
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, The Ministry of Education Key Laboratory, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, China
| | - Li Wang
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, The Ministry of Education Key Laboratory, National Clinical Research Center for Dermatologic and Immunologic Diseases, No. 1 Shuaifuyuan, Dongcheng District, Beijing 100730, China
| | - Yongtai Liu
- Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
| | - Fengchun Zhang
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, The Ministry of Education Key Laboratory, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing 100730, China
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Pazzola G, Pipitone N, Salvarani C. Cardiac involvement in the adult primary vasculitides. Expert Rev Clin Immunol 2020; 16:985-991. [PMID: 32954889 DOI: 10.1080/1744666x.2021.1823219] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/23/2022]
Abstract
INTRODUCTION Heart involvement in vasculitis is rare, but potentially severe. The ascertainment of cardiac disease in vasculitis is complex and requires an integrated multidisciplinary approach involving the Rheumatologist, Radiologist, Cardiologist, and Heart surgeon. AREAS COVERED the authors searched PubMed using the keywords 'heart'[Mesh] and vasculitis"[Mesh]. EXPERT OPINION Virtually any vasculitis can affect the heart, but cardiac involvement is more common in some vasculitides such as Takayasu arteritis, polyarteritis nodosa, and eosinophilic granulomatosis with polyangiitis. Immunosuppressive treatment and when indicated surgery can improve the prognosis.
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Affiliation(s)
- Giulia Pazzola
- Rheumatology Unit, Department of Internal Medicine, Azienda USL-IRCCS di Reggio Emilia e Università di Modena e Reggio Emilia , Reggio Emilia, Italy
| | - Nicolò Pipitone
- Rheumatology Unit, Department of Internal Medicine, Azienda USL-IRCCS di Reggio Emilia e Università di Modena e Reggio Emilia , Reggio Emilia, Italy
| | - Carlo Salvarani
- Rheumatology Unit, Department of Internal Medicine, Azienda USL-IRCCS di Reggio Emilia e Università di Modena e Reggio Emilia , Reggio Emilia, Italy
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Feng Y, Wang P, Cui B. Usefulness of PET/CT in ANCA associated renal vasculitis. Med Clin (Barc) 2020; 155:185-186. [DOI: 10.1016/j.medcli.2019.05.038] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/28/2019] [Revised: 05/10/2019] [Accepted: 05/16/2019] [Indexed: 10/26/2022]
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Li C, Zou Y, Lu X, Wang G, Shu X. Pituitary dysfunction in patients with ANCA associated vasculitis: prevalence, presentation, and outcomes. Ther Adv Chronic Dis 2020; 11:2040622320930636. [PMID: 32566116 PMCID: PMC7285954 DOI: 10.1177/2040622320930636] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/20/2019] [Accepted: 04/22/2020] [Indexed: 11/23/2022] Open
Abstract
Background: The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are rare multisystem autoimmune diseases characterized by inflammatory cell infiltration causing necrosis of small blood vessels. Pituitary involvement in AAV is poorly described. This study aimed to describe the prevalence, clinical characteristics, and outcomes of pituitary involvement in patients with AAV. Methods: A total of 150 patients diagnosed with AAV and hospitalized in the China–Japan Friendship Hospital between 2009 and 2019 were enrolled in this retrospective study. Patients diagnosed with pituitary involvement in AAV were selected for inclusion. Results: Three patients (2%) were identified with pituitary involvement. Two patients had positive ANCA titers, one with proteinase 3 positive and one with myeloperoxidase positive antibodies. Pituitary dysfunction presented as an initial symptom in one patient and developed over the course of the diseases in the other two patients. All three patients had abnormal hormones. Among them, two patients had an enlarged pituitary, shown by magnetic resonance images (MRIs), and one patient had a normal sized pituitary, shown by MRI, but presented with increased linear radioactivity uptake in the pituitary fossa by positron emission tomography-computed tomography. All patients were treated with corticosteroid and immunosuppressive therapy. Both pituitary dysfunction and vasculitis were in remission. Conclusion: Pituitary involvement is uncommon in AAV and it can occur at any point during AAV. The main clinical manifestations are central diabetes insipidus and panhypopituitarism. Immunosuppressive therapy could significantly alleviate clinical symptoms as well as pituitary imaging.
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Affiliation(s)
- Chunjia Li
- Department of Rheumatology, China-Japan Friendship Hospital, Beijing, China
| | - Yu Zou
- Department of Rheumatology, China-Japan Friendship Hospital, Beijing, China
| | - Xin Lu
- Department of Rheumatology, China-Japan Friendship Hospital, Beijing, China
| | - Guochun Wang
- Department of Rheumatology, China-Japan Friendship Hospital, Beijing, China
| | - Xiaoming Shu
- Department of Rheumatology, China-Japan Friendship Hospital, Yinghua East Road, Chaoyang District, Beijing, 100029, China
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Solans-Laqué R, Rodriguez-Carballeira M, Rios-Blanco JJ, Fraile G, Sáez-Comet L, Martinez-Zapico A, Frutos B, Solanich X, Fonseca-Aizpuru E, Pasquau-Liaño F, Zamora M, Oristrell J, Fanlo P, Lopez-Dupla M, Abdilla M, García-Sánchez I, Sopeña B, Castillo MJ, Perales I, Callejas JL. Comparison of the Birmingham Vasculitis Activity Score and the Five-Factor Score to Assess Survival in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A Study of 550 Patients From Spain (REVAS Registry). Arthritis Care Res (Hoboken) 2020; 72:1001-1010. [PMID: 31033198 DOI: 10.1002/acr.23912] [Citation(s) in RCA: 23] [Impact Index Per Article: 4.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/05/2018] [Accepted: 04/23/2019] [Indexed: 01/26/2023]
Abstract
OBJECTIVE To compare the accuracy of the Birmingham Vasculitis Activity Score (BVAS), version 3, and the Five Factor Score (FFS), version 1996 and version 2009, to assess survival in antineutrophil cytoplasmic antibody-associated vasculitis (AAV). METHODS A total of 550 patients with AAV (41.1% with granulomatosis with polyangiitis, 37.3% with microscopic polyangiitis, and 21.6% with eosinophilic granulomatosis with polyangiitis), diagnosed between 1990 and 2016, were analyzed. Receiver operating characteristic (ROC) curves and multivariable Cox analysis were used to assess the relationships between the outcome and the different scores. RESULTS Overall mortality was 33.1%. The mean ± SD BVAS at diagnosis was 17.96 ± 7.82 and was significantly higher in nonsurvivors than in survivors (mean ± SD 20.0 ± 8.14 versus 16.95 ± 7.47, respectively; P < 0.001). The mean ± SD 1996 FFS and 2009 FFS were 0.81 ± 0.94 and 1.47 ± 1.16, respectively, and were significantly higher in nonsurvivors than in survivors (mean ± SD 1996 FFS 1.17 ± 1.07 versus 0.63 ± 0.81 [P < 0.001] and 2009 FFS 2.13 ± 1.09 versus 1.15 ± 1.05 [P < 0.001], respectively). Mortality rates increased according to the different 1996 FFS and 2009 FFS categories. In multivariate analysis, BVAS, 1996 FFS, and 2009 FFS were significantly related to death (P = 0.007, P = 0.020, P < 0.001, respectively), but the stronger predictor was the 2009 FFS (hazard ratio 2.9 [95% confidence interval 2.4-3.6]). When the accuracy of BVAS, 1996 FFS, and 2009 FFS to predict survival was compared in the global cohort, ROC analysis yielded area under the curve values of 0.60, 0.65, and 0.74, respectively, indicating that 2009 FFS had the best performance. Similar results were obtained when comparing these scores in patients diagnosed before and after 2001 and when assessing the 1-year, 5-year, and long-term mortality. Correlation among BVAS and 1996 FFS was modest (r = 0.49; P < 0.001) but higher than between BVAS and the 2009 FFS (r = 0.28; P < 0.001). CONCLUSION BVAS and FFS are useful to predict survival in AAV, but the 2009 FFS has the best prognostic accuracy at any point of the disease course.
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Ludwig DR, Amin TN, Manson JJ. Suspected systemic rheumatic diseases in adults presenting with fever. Best Pract Res Clin Rheumatol 2019; 33:101426. [DOI: 10.1016/j.berh.2019.06.008] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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Přikryl P, Hrušková Z, Konopásek P, Hladinová Z, Tesař V, Vokurka M. Serum hepcidin is increased in ANCA-associated vasculitis and correlates with activity markers. Physiol Res 2018; 67:945-954. [PMID: 30204470 DOI: 10.33549/physiolres.933765] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022] Open
Abstract
Hepcidin is a key regulator of iron metabolism and plays an important role in many pathologies. It is increased by iron administration and by inflammation, while erythropoiesis downregulates its expression. It decreases iron availability and thus contributes to anemia of chronic diseases. The aim of the study was to measure hepcidin as a marker and pathogenetic factor in ANCA-associated vasculitis (AAV). Hepcidin plasma concentration was measured by the immunological method in 59 patients with AAV and compared to patients with non-vasculitic etiology of chronic kidney disease, patients on hemodialysis (HD), with systemic lupus erythematodes (SLE) and to healthy controls and blood donors, and was correlated with the parameters of iron metabolism, inflammation, activity of the process and kidney function. Hepcidin concentration was increased in patients with AAV, SLE and HD and correlated positively with C-reactive protein, serum ferritin and creatinine, and negatively with hemoglobin and serum transferrin. In active form of AAV it correlated with the clinical scoring system (BVAS). Hepcidin can thus be considered as a pathogenetic factor of anemia in AAV and can be used for evaluation of inflammation in AAV and as an additional marker in active forms of the disease.
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Affiliation(s)
- P Přikryl
- Institute of Pathological Physiology, First Faculty of Medicine, Charles University, Prague, Czech Republic, Department of Nephrology, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic. and
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Graf J. Central Nervous System Disease in Antineutrophil Cytoplasmic Antibodies–Associated Vasculitis. Rheum Dis Clin North Am 2017; 43:573-578. [DOI: 10.1016/j.rdc.2017.06.006] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022]
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Solans-Laqué R, Fraile G, Rodriguez-Carballeira M, Caminal L, Castillo MJ, Martínez-Valle F, Sáez L, Rios JJ, Solanich X, Oristrell J, Pasquau F, Fonseca E, Zamora M, Callejas JL, Frutos B, Abdilla M, Fanlo P, García-Sánchez I, López-Dupla M, Sopeña B, Pérez-Iglesias A, Bosch JA. Clinical characteristics and outcome of Spanish patients with ANCA-associated vasculitides: Impact of the vasculitis type, ANCA specificity, and treatment on mortality and morbidity. Medicine (Baltimore) 2017; 96:e6083. [PMID: 28225490 PMCID: PMC5569416 DOI: 10.1097/md.0000000000006083] [Citation(s) in RCA: 97] [Impact Index Per Article: 12.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/22/2023] Open
Abstract
The aim of this study was to describe the clinical characteristics of ANCA-associated vasculitides (AAV) at presentation, in a wide cohort of Spanish patients, and to analyze the impact of the vasculitis type, ANCA specificity, prognostic factors, and treatments administered at diagnosis, in the outcome.A total of 450 patients diagnosed between January 1990 and January 2014 in 20 Hospitals from Spain were included. Altogether, 40.9% had granulomatosis with polyangiitis (GPA), 37.1% microscopic polyangiitis (MPA), and 22% eosinophilic granulomatosis with polyangiitis (EGPA). The mean age at diagnosis was 55.6 ± 17.3 years, patients with MPA being significantly older (P < 0.001). Fever, arthralgia, weight loss, respiratory, and ear-nose-throat (ENT) symptoms, were the most common at disease onset. ANCAs tested positive in 86.4% of cases: 36.2% C-ANCA-PR3 and 50.2% P-ANCA-MPO. P-ANCA-MPO was significantly associated with an increased risk for renal disease (OR 2.6, P < 0.001) and alveolar hemorrhage (OR 2, P = 0.010), while C-ANCA-PR3 was significantly associated with an increased risk for ENT (OR 3.4, P < 0.001) and ocular involvement (OR 2.3, P = 0.002). All patients received corticosteroids (CS) and 74.9% cyclophosphamide (CYC). The median follow-up was 82 months (IQR 100.4). Over this period 39.9% of patients suffered bacterial infections and 14.6% opportunistic infections, both being most prevalent in patients with high-cumulated doses of CYC and CS (P < 0.001). Relapses were recorded in 36.4% of cases with a mean rate of 2.5 ± 2.3, and were more frequent in patients with C-ANCA-PR3 (P = 0.012). The initial disease severity was significantly associated with mortality but not with the occurrence of relapses. One hundred twenty-nine (28.7%) patients (74 MPA, 41 GPA, 14 EGPA) died. The mean survival was 58 months (IQR 105) and was significantly lower for patients with MPA (P < 0.001). Factors independently related to death were renal involvement (P = 0.010), cardiac failure (P = 0.029) and age over 65 years old (P < 0.001) at disease onset, and bacterial infections (P < 0.001). An improved outcome with significant decrease in mortality and treatment-related morbidity was observed in patients diagnosed after 2000, and was related to the implementation of less toxic regimens adapted to the disease activity and stage, and a drastic reduction in the cumulated CYC and CS dose.
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Li ZY, Ma TT, Chen M, Zhao MH. The Prevalence and Management of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis in China. KIDNEY DISEASES (BASEL, SWITZERLAND) 2016; 1:216-23. [PMID: 27536681 PMCID: PMC4934806 DOI: 10.1159/000441912] [Citation(s) in RCA: 25] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 09/23/2015] [Accepted: 10/18/2015] [Indexed: 11/19/2022]
Abstract
BACKGROUND Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) comprises microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA). Myeloperoxidase (MPO) and proteinase 3 (PR3) are the main antigens for ANCA. AAV is a common multisystem autoimmune disease and most of the studies on AAV have been conducted in Western countries. Nowadays in China many efforts are made to investigate this disease. SUMMARY This review highlights the progress in the prevalence, management and outcomes of AAV in Chinese patients. With respect to the prevalence of AAV, though there are no precise data, AAV is not rare in the Chinese population. In Chinese patients with AAV there is a striking preponderance of MPA, and MPO-ANCA is much more common than PR3-ANCA. Even in patients with GPA there is a predominance of MPO-ANCA over PR3-ANCA. Propylthiouracil-induced AAV and ANCA-negative pauci-immune glomerulonephritis are stated in this review as well. With respect to the management of AAV, glucocorticoids in combination with cyclophosphamide remain the mainstay of induction therapy. Besides, we describe predictors of different outcomes in Chinese patients, including mortality, relapse, treatment resistance and end-stage renal disease. KEY MESSAGES AAV is not rare in the Chinese population. The disease spectrum and subtypes of ANCA are different between patients with AAV in China and Western countries. The treatment strategy for AAV in China is in consistency with that in Western countries. Predictors of different clinical outcomes are provided. FACTS FROM EAST AND WEST Treatment options for AAV are shared between the East and West, with corticosteroid combined with cyclophosphamide being the standard regimen for inductive therapy and switching to azathioprine after remission. The major cause of death in treated patients is infection related to immunosuppressive therapy within the first year after diagnosis, and this rate might be higher in China than in Western countries. Western studies demonstrated the efficacy and safety of rituximab for induction of remission in cases with relatively mild disease and maintenance therapy, but this agent is rarely used in China.
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Affiliation(s)
- Zhi-Ying Li
- Renal Division, Department of Medicine, Peking University First Hospital, Beijing, China
- Peking University Institute of Nephrology, Beijing, China
- Peking-Tsinghua Center for Life Sciences, Beijing, China
- Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China
- Key Laboratory of Chronic Kidney Disease Prevention and Treatment (Peking University), Ministry of Education, Beijing, China
| | - Tian-Tian Ma
- Renal Division, Department of Medicine, Peking University First Hospital, Beijing, China
- Peking University Institute of Nephrology, Beijing, China
- Peking-Tsinghua Center for Life Sciences, Beijing, China
- Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China
- Key Laboratory of Chronic Kidney Disease Prevention and Treatment (Peking University), Ministry of Education, Beijing, China
| | - Min Chen
- Renal Division, Department of Medicine, Peking University First Hospital, Beijing, China
- Peking University Institute of Nephrology, Beijing, China
- Peking-Tsinghua Center for Life Sciences, Beijing, China
- Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China
- Key Laboratory of Chronic Kidney Disease Prevention and Treatment (Peking University), Ministry of Education, Beijing, China
| | - Ming-Hui Zhao
- Renal Division, Department of Medicine, Peking University First Hospital, Beijing, China
- Peking University Institute of Nephrology, Beijing, China
- Peking-Tsinghua Center for Life Sciences, Beijing, China
- Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China
- Key Laboratory of Chronic Kidney Disease Prevention and Treatment (Peking University), Ministry of Education, Beijing, China
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Misra DP, Shenoy SN. Cardiac involvement in primary systemic vasculitis and potential drug therapies to reduce cardiovascular risk. Rheumatol Int 2016; 37:151-167. [DOI: 10.1007/s00296-016-3435-1] [Citation(s) in RCA: 36] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/12/2015] [Accepted: 01/26/2016] [Indexed: 12/13/2022]
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Usuda D, Sangen R, Iwata Y, Kanda T. Interstitial Pneumonia in a 94-Year-Old Woman with MPO-ANCA Positive Vasculitis. CASE REPORTS IN CLINICAL MEDICINE 2016. [DOI: 10.4236/crcm.2016.52011] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
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