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Qian Y, Lu M, Zheng Q. IL-10 and IFN-γ as markers for early recognition of pediatric systemic lupus erythematosus complicated with macrophage activation syndrome. Rheumatology (Oxford) 2025; 64:235-241. [PMID: 38109672 DOI: 10.1093/rheumatology/kead678] [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: 08/05/2023] [Revised: 11/09/2023] [Accepted: 11/27/2023] [Indexed: 12/20/2023] Open
Abstract
OBJECTIVES To compare the clinical and laboratory characteristics of pediatric-onset systemic lupus erythematosus (pSLE), pSLE with macrophage activation syndrome (MAS), and pSLE with recurrent MAS, and to find biomarkers for the differential diagnosis of these diseases. METHODS Demographic, clinical, laboratory and radiological data were analysed for three groups of patients: 18 cases of pSLE with MAS, 48 age- and sex-matched cases of active pSLE without MAS and 40 age- and sex-matched cases of pSLE with inactive disease. One case of a 9-year-old girl with recurrent MAS as the primary manifestation of SLE also was recorded. RESULTS IL-10 and IFN-γ levels were significantly higher in pSLE patients with MAS than in pSLE patients without MAS, and were significantly correlated with SLE and MAS laboratory features. Levels of IL-10 > 7.25 pg/ml had a high sensitivity and levels of IFN-γ > 6.7 pg/ml had a high specificity for predicting MAS in pSLE. Constitutional symptoms were evident in the case of recurrent MAS in pSLE, and traditional immunosuppressive therapies were unable to prevent the next MAS episode. CONCLUSION Compared with pSLE and pSLE-MAS with a single episode, pSLE with recurrent MAS has different clinical manifestations and responses to treatment, requiring intensive studies to elucidate the underlying pathogenic mechanisms. Elevated serum levels of IL-10 and IFN-γ may be correlated with pSLE with MAS and can serve as serum biomarkers for pSLE with MAS.
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Affiliation(s)
- Yanjie Qian
- Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China
| | - Meiping Lu
- Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China
- Department of Rheumatology, Immunology and Allergy, Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China
| | - Qi Zheng
- Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China
- Department of Rheumatology, Immunology and Allergy, Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China
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Chen S, Zhang C, Luo J, Lin Z, Chang T, Dong L, Chen D, Tang ZH. Macrophage activation syndrome in Sepsis: from pathogenesis to clinical management. Inflamm Res 2024; 73:2179-2197. [PMID: 39404874 DOI: 10.1007/s00011-024-01957-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/20/2024] [Revised: 08/01/2024] [Accepted: 10/01/2024] [Indexed: 12/11/2024] Open
Abstract
BACKGROUND Sepsis represents a significant global health and hygiene challenge. Excessive activation of macrophages in sepsis can result in certain patients displaying characteristics akin to those observed in Macrophage Activation Syndrome (MAS). MAS represents a grave immune system disorder characterized by persistent and severe inflammation within the body. In the context of sepsis, MAS presents atypically, leading some researchers to refer to it as Macrophage Activation-Like Syndrome (MALS). However, there are currently no effective treatment measures for this situation. The purpose of this article is to explore potential treatment methods for sepsis-associated MALS. OBJECTIVE The objective of this review is to synthesize the specific pathophysiological mechanisms and treatment strategies of MAS to investigate potential therapeutic approaches for sepsis-associated MALS. METHOD We searched major databases (including PubMed, Web of Science, and Google Scholar etc.) for literature encompassing macrophage activation syndrome and sepsis up to Mar 2024 and combined with studies found in the reference lists of the included studies. CONCLUSION We have synthesized the underlying pathophysiological mechanism of MALS in sepsis, and then summarized the diagnostic criteria and the effects of various treatment modalities utilized in patients with MAS or MALS. In both scenarios, heterogeneous treatment responses resulting from identical treatment approaches were observed. The determination of whether the patient is genuinely experiencing MALS significantly impacts the ultimate outcomes of therapeutic efficacy. In order to tackle this concern, additional clinical trials and research endeavors are imperative.
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Affiliation(s)
- Shunyao Chen
- Department of Trauma Surgery, Emergency Surgery & Surgical Critical, Tongji Trauma Center, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
- Department of Emergency and Critical Care Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China
| | - Cong Zhang
- Department of Trauma Surgery, Emergency Surgery & Surgical Critical, Tongji Trauma Center, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
- Department of Emergency and Critical Care Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China
| | - Jialiu Luo
- Department of Trauma Surgery, Emergency Surgery & Surgical Critical, Tongji Trauma Center, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
- Department of Emergency and Critical Care Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China
| | - Zhiqiang Lin
- Department of Trauma Surgery, Emergency Surgery & Surgical Critical, Tongji Trauma Center, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
- Department of Emergency and Critical Care Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China
| | - Teding Chang
- Department of Trauma Surgery, Emergency Surgery & Surgical Critical, Tongji Trauma Center, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
- Department of Emergency and Critical Care Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China
| | - Liming Dong
- Department of Trauma Surgery, Emergency Surgery & Surgical Critical, Tongji Trauma Center, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
- Department of Emergency and Critical Care Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China.
| | - Deng Chen
- Department of Trauma Surgery, Emergency Surgery & Surgical Critical, Tongji Trauma Center, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
- Department of Emergency and Critical Care Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China.
| | - Zhao-Hui Tang
- Department of Trauma Surgery, Emergency Surgery & Surgical Critical, Tongji Trauma Center, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
- Department of Emergency and Critical Care Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China.
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Machado T, Lawrence J, Eriksson D, Donohue J. A need for greater awareness: a mixed methods study of patient and healthcare professional perspectives on the diagnosis journey for haemophagocytic lymphohistiocytosis (HLH). Hematology 2024; 29:2358261. [PMID: 38934707 DOI: 10.1080/16078454.2024.2358261] [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: 08/04/2023] [Accepted: 05/14/2024] [Indexed: 06/28/2024] Open
Abstract
OBJECTIVES Our aim was to better understand and raise awareness of the diagnosis journey and quantify any barriers for timely diagnosis of haemophagocytic lymphohistiocytosis (HLH), to support patients' struggle with diagnosis and reduce time to diagnosis. METHODS Patients diagnosed with, or caregivers for those diagnosed with primary or secondary HLH and physicians involved in the treatment of HLH were recruited. Quantitative interviews were undertaken with patients/caregivers to quantify key elements of the diagnosis journey, followed by qualitative interviews with participants. Interviews took place between March-May 2021. RESULTS Thirty-three patients/caregivers and nine physicians took part in this mixed methods study. Lack of physician awareness of HLH was a common frustration for patients/caregivers, causing delayed diagnosis. All physicians indicated bone-marrow testing is a key step in the diagnosis process, and some patients/caregivers had frustrations around testing. Emergency care doctors, although not usually involved in the diagnosis process, were among the most-seen specialists by patients/caregivers. Patients/caregivers suggested potential improvements in available information, such as providing information on treatment options and condition management. DISCUSSION Patients/caregivers and physicians agreed on the need to raise overall awareness of HLH signs/symptoms among priority groups of physicians to recognise how signs/symptoms can progress and develop. Improvements in the testing process and communication would directly impact the speed of diagnosis and support patients/caregivers during the diagnostic journey, respectively. CONCLUSION Raising awareness of key issues, such as signs/symptoms, tests and diagnostic procedures, and improved communication and support for patients/caregivers, are key to speeding up HLH diagnosis and improving outcomes.
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Ramadoss I, Rengabashyam P, Seetharaman Varadhan M, Ponniah Subramanian AR. Macrophage activation syndrome as a presenting feature in juvenile systemic lupus erythematosus. Lupus 2024; 33:1254-1259. [PMID: 39109623 DOI: 10.1177/09612033241272972] [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] [Indexed: 09/19/2024]
Abstract
BACKGROUND Macrophage activation syndrome (MAS) is an acquired form of hemo phagocytic lymphohistiocytosis (HLH) and is usually associated with infections, autoimmune, auto inflammatory syndromes and malignancies. CASE DETAILS A 14 year old girl presented with sub-acute onset of fever with lymphadenopathy, pancytopenia,high ferritin values and a falling erythrocyte sedimentation rate. She was evaluated with relevant laboratory tests that was suggestive of systemic Lupus erythematosus and associated macrophage activation syndrome She recovered with immunosuppressive therapy and other supportive care. CONCLUSION There is a need for a high index of suspicion of occult MAS and MAS in patients with systemic lupus erythematosus as it may be an initial presentation. Delay in diagnosis and initiation of treatment can lead to a higher mortality.
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Affiliation(s)
- Ishwarya Ramadoss
- Institute of Rheumatology, Rajiv Gandhi Government General Hospital, Madras Medical College, Chennai, India
| | - Pirahalathan Rengabashyam
- Institute of Rheumatology, Rajiv Gandhi Government General Hospital, Madras Medical College, Chennai, India
| | | | - Arul R Ponniah Subramanian
- Institute of Rheumatology, Rajiv Gandhi Government General Hospital, Madras Medical College, Chennai, India
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Wang J, Rong W, Yan H. Eighty-six cases of clinical characteristics and outcomes of systemic lupus erythematosus-associated macrophage activation syndrome: A meta-analysis study. Immun Inflamm Dis 2024; 12:e1364. [PMID: 39110110 PMCID: PMC11304897 DOI: 10.1002/iid3.1364] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/03/2023] [Revised: 06/28/2024] [Accepted: 07/23/2024] [Indexed: 08/10/2024] Open
Abstract
OBJECTIVE To improve our understanding of systemic lupus erythematosus (SLE)-macrophage activation syndrome (MAS). METHODS A systematic review was performed, to retrieve all those papers on patients with SLE-MAS, in individual or aggregated form. The data in each of these medical records were extracted and analyzed to identify the characteristics of SLE-MAS. RESULTS A total of 86 SLE-MAS patients were included (25 males and 61 females. The mean (±standard error of the mean) age was 31.21 ± 1.694 years. MAS occurred as the initial presentation of SLE in 47 people (54.65%) and during the course of SLE in 39 (45.35%). A coinfection was reported in 23 (26.74%) patients. The mean Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) score was 16.54 ± 0.9462. Overall, 10 patients (11.63%) died. The SLEDAI-2K score was higher in patients with MAS as an initial manifestation of SLE than in those where MAS occurred during the course of SLE. The proportion of patients receiving steroid pulse therapy was lower in patients with coinfections. The deceased group demonstrated lower platelet and ferritin levels. Multiple regression analysis revealed that age and thrombocytopenia were independent factors associated with poor prognosis. In receiver operating characteristic analysis, a platelet count cutoff value of ≤47 × 109/L was a predictor of poor outcome. CONCLUSIONS SLE-MAS patients demonstrated high lupus activity, and lupus activity was especially higher in patients with MAS as an initial manifestation. Lupus activity was the predominant trigger of lupus MAS. Thrombocytopenia was an independent factor for poor prognosis.
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Affiliation(s)
- Jingya Wang
- Department of Rheumatology, Wuhu HospitalEast China Normal University (The Second People's Hospital of Wuhu)WuhuAnhuiChina
| | - Wei Rong
- Department of Rheumatology, Wuhu HospitalEast China Normal University (The Second People's Hospital of Wuhu)WuhuAnhuiChina
| | - Haotian Yan
- Department of Rheumatology, Wuhu HospitalEast China Normal University (The Second People's Hospital of Wuhu)WuhuAnhuiChina
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Kalashnikova E, Isupova E, Gaidar E, Sorokina L, Kaneva M, Masalova V, Dubko M, Kornishina T, Lubimova N, Kuchinskaya E, Chikova I, Raupov R, Kalashnikova O, Kostik M. BCD020 rituximab bioanalog compared to standard treatment in juvenile systemic lupus erythematosus: The data of 12 months case-control study. World J Clin Pediatr 2024; 13:89049. [PMID: 38596443 PMCID: PMC11000064 DOI: 10.5409/wjcp.v13.i1.89049] [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: 10/19/2023] [Revised: 02/02/2024] [Accepted: 01/30/2024] [Indexed: 03/06/2024] Open
Abstract
BACKGROUND Systemic lupus erythematosus (SLE) is the most frequent and serious systemic connective tissue disease. Nowadays there is no clear guidance on its treatment in childhood. There are a lot of negative effects of standard-of-care treatment (SOCT), including steroid toxicity. Rituximab (RTX) is the biological B-lymphocyte-depleting agent suggested as a basic therapy in pediatric SLE. AIM To compare the benefits of RTX above SOCT. METHODS The data from case histories of 79 children from the Saint-Petersburg State Pediatric Medical University from 2012 to 2022 years, were analyzed. The diagnosis of SLE was established with SLICC criteria. We compared the outcomes of treatment of SLE in children treated with and without RTX. Laboratory data, doses of glucocorticosteroids, disease activity measured with SELENA-SLEDAI, and organ damage were assessed at the time of initiation of therapy and one year later. RESULTS Patients, treated with RTX initially had a higher degree of disease activity with prevalence of central nervous system and kidney involvement, compared to patients with SOCT. One year later the disease characteristics became similar between groups with a more marked reduction of disease activity (SELENA-SLEDAI activity index) in the children who received RTX [-19 points (17; 23) since baseline] compared to children with SOCT [-10 (5; 15.5) points since baseline, P = 0.001], the number of patients with active lupus nephritis, and daily proteinuria. During RTX therapy, infectious diseases had three patients; one patient developed a bi-cytopenia. CONCLUSION RTX can be considered as the option in the treatment of severe forms of SLE, due to its ability to arrest disease activity compared to SOCT.
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Affiliation(s)
- Elvira Kalashnikova
- Hospital Pediatry Department, Saint-Petersburg State Pediatric Medical University, Saint-Petersburg 194100, Russia
| | - Eugenia Isupova
- Hospital Pediatry Department, Saint-Petersburg State Pediatric Medical University, Saint-Petersburg 194100, Russia
| | - Ekaterina Gaidar
- Hospital Pediatry Department, Saint-Petersburg State Pediatric Medical University, Saint-Petersburg 194100, Russia
| | - Lyubov Sorokina
- Hospital Pediatry Department, Saint-Petersburg State Pediatric Medical University, Saint-Petersburg 194100, Russia
| | - Maria Kaneva
- Hospital Pediatry Department, Saint-Petersburg State Pediatric Medical University, Saint-Petersburg 194100, Russia
| | - Vera Masalova
- Hospital Pediatry Department, Saint-Petersburg State Pediatric Medical University, Saint-Petersburg 194100, Russia
| | - Margarita Dubko
- Hospital Pediatry Department, Saint-Petersburg State Pediatric Medical University, Saint-Petersburg 194100, Russia
| | - Tatiana Kornishina
- Hospital Pediatry Department, Saint-Petersburg State Pediatric Medical University, Saint-Petersburg 194100, Russia
| | - Natalia Lubimova
- Research Laboratory of Autoimmune and Autoinflammatory Diseases, Almazov National Medical Research Centre, Saint Petersburg 197341, Russia
| | - Ekaterina Kuchinskaya
- Research Laboratory of Autoimmune and Autoinflammatory Diseases, Almazov National Medical Research Centre, Saint Petersburg 197341, Russia
| | - Irina Chikova
- Hospital Pediatry Department, Saint-Petersburg State Pediatric Medical University, Saint-Petersburg 194100, Russia
| | - Rinat Raupov
- Hospital Pediatry Department, Saint-Petersburg State Pediatric Medical University, Saint-Petersburg 194100, Russia
- Department of Rheumatology, Turner National Medical Research Center for Сhildren’s Orthopedics and Trauma Surgery, Saint-Petetrsburg 197136, Russia
| | - Olga Kalashnikova
- Hospital Pediatry Department, Saint-Petersburg State Pediatric Medical University, Saint-Petersburg 194100, Russia
| | - Mikhail Kostik
- Hospital Pediatry Department, Saint-Petersburg State Pediatric Medical University, Saint-Petersburg 194100, Russia
- Research Laboratory of Autoimmune and Autoinflammatory Diseases, Almazov National Medical Research Centre, Saint Petersburg 197341, Russia
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Velayutham B, Padhi S, Devi S, Patra S, Panigrahi C, Ramasubbu MK, Kumar R, Raheman S. Immunohistochemical expression of perforin in adult systemic lupus erythematosus associated macrophage activation syndrome: Clinicohematological correlation and literature review. Lupus 2024; 33:26-39. [PMID: 38069452 DOI: 10.1177/09612033231221414] [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] [Indexed: 12/21/2023]
Abstract
OBJECTIVE To study the bone marrow (BM) immunohistomorphological characteristics in adult systemic lupus erythematosus (SLE) associated macrophage activation syndrome (SLE-MAS). MATERIALS AND METHODS Immunohistochemical (IHC) expression of CD3, CD8, perforin (PFN), and CD163 was studied on BM trephine biopsies from 30 cytopenic adult SLE cases (male: female = 1:5, age; 24 years, range; 19-32) and compared them with ten age matched controls. Clinicopathological parameters were compared among the cases likely (L) or unlikely (U) to have MAS using probability scoring criteria. The best cut off laboratory parameters to discriminate between the two were obtained through receiver operator curve (ROC) analysis. RESULTS MAS occurred in 12/30 (40%) cases and was more commonly associated with prior immunosuppressive therapy (p = .07), ≥ 3 system involvement (p = .09), lower fibrinogen (p < .01), increased triglyceride (p = .002), increased BM hemophagocytosis (p = .002), and higher MAS score [185 (176-203) vs. 105 (77-119), p < .01] than MAS-U subgroup. Although PFN+CD8+ T lymphocytes significantly decreased among cases than controls (p < .05), it was comparable between MAS-L and MAS-U subgroups. Fibrinogen (< 2.4 g/L, AUC; 0.93, p < .01), hemophagocytosis score (> 1.5, AUC; 0.71, p = .03), and an MAS probability score of ≥ 164 (AUC; 1, p < .01) discriminated MAS from those without MAS. CONCLUSION We noted a decrease in perforin mediated CD8 + T cell cytotoxicity in SLE. Immunohistochemical demonstration of the same along with histiocytic hemophagocytosis on BM biopsy may be useful adjunct in early diagnosis and management of MAS in SLE.
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Affiliation(s)
- Bakialakshmi Velayutham
- Department of Pathology with Laboratory Medicine, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
| | - Somanath Padhi
- Department of Pathology with Laboratory Medicine, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
| | - Sujata Devi
- Department of General Medicine, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
| | - Susama Patra
- Department of Pathology with Laboratory Medicine, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
| | - Chinmayee Panigrahi
- Department of Pathology with Laboratory Medicine, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
| | - Mathan Kumar Ramasubbu
- Department of Pharmacology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
| | - Rajesh Kumar
- Department of General Medicine, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
- Department of General Medicine, All India Institute of Medical Sciences, Deoghar, Jharkhand, India
| | - Samiur Raheman
- Department of Pathology with Laboratory Medicine, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
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Ebrahimi M, Aghapour SA, Rashidbaghan A, Mazandarani M. Stevens-Johnson syndrome and COVID-19: a case report with suspected multisystem inflammatory syndrome in children (MIS-C). Ann Med Surg (Lond) 2023; 85:5641-5644. [PMID: 37915708 PMCID: PMC10617869 DOI: 10.1097/ms9.0000000000001087] [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: 11/03/2022] [Accepted: 07/09/2023] [Indexed: 11/03/2023] Open
Abstract
Introduction and importance Symptoms similar to diseases such as Stevens-Johnson syndrome (SJS) and multisystemic inflammatory syndrome in children (MIS-C) were reported in pediatric coronavirus infections. Case presentation Here, we present a 4-year-old girl with coronavirus disease 2019 (COVID-19), an earlier diagnosis of SJS, and a final diagnosis of MIS-C. Clinical discussion Unlike the negative PCR test for severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2), the positive serological test confirmed COVID-19. Conclusion The monitoring of this case indicated that higher coronavirus infection can delay immune reaction and cause symptoms similar to SJS.
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Affiliation(s)
| | - Seyed Ali Aghapour
- Neonatal and Children’s Health Research Center, Golestan University of Medical Sciences, Gorgan, Iran
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Mercader-Salvans J, García-González M, Quevedo-Abeledo JC, Quevedo-Rodríguez A, Romo-Cordero A, Ojeda-Bruno S, Gómez-Bernal F, López-Mejías R, Martín-González C, González-Gay MÁ, Ferraz-Amaro I. Blood Composite Scores in Patients with Systemic Lupus Erythematosus. Biomedicines 2023; 11:2782. [PMID: 37893155 PMCID: PMC10604879 DOI: 10.3390/biomedicines11102782] [Citation(s) in RCA: 6] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/29/2023] [Revised: 09/16/2023] [Accepted: 10/10/2023] [Indexed: 10/29/2023] Open
Abstract
Complete blood count-derived ratios have been described as inflammatory biomarkers in several diseases. These hematological scores include the neutrophil-to-lymphocyte ratio (NLR), monocyte-to-lymphocyte ratio (MLR), platelet-to-lymphocyte ratio (PLR), and systemic immune-inflammatory index ([SIRI]; neutrophils × monocytes/lymphocytes). Our aim was to study how these biomarkers are related to disease expression in a large and well-characterized series of patients with systemic lupus erythematosus (SLE). A total of 284 SLE patients and 181 age- and sex-matched healthy controls were recruited. The NLR, MLR, PLR, and SIRI were calculated, and activity (SLEDAI-2K), severity (Katz), and damage index (SLICC-DI) scores were assessed in patients with SLE. Multivariable linear regression analysis was performed to study whether these scores differ between patients and controls and how they are related to clinical and laboratory features of the disease. Crude cell counts of neutrophils, monocytes, lymphocytes, and platelets were lower in SLE patients compared to controls. Despite this, NLR, MLR, and PRL, but not SIRI, were higher in SLE patients than in controls after multivariable analysis. However, the relationship between the different scores and disease characteristics was limited. Only the Katz severity index revealed a significant positive relationship with SIRI, NLR, and MLR after adjustment for covariates. Similarly, alternative complement cascade activation and low C3 were significantly associated with higher NLR, MLR, and PLR. In conclusion, although cytopenias are a common feature of patients with SLE, hematologic composite scores are independently higher in this population compared to controls. However, the relationship of these scores with the characteristics of the disease is scarce, with the relationship with the complement system being the most consistent.
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Affiliation(s)
| | - María García-González
- Division of Rheumatology, Hospital Universitario de Canarias, 38320 Tenerife, Spain;
| | - Juan C. Quevedo-Abeledo
- Division of Rheumatology, Hospital Doctor Negrín, 35010 Las Palmas de Gran Canaria, Spain; (J.C.Q.-A.); (A.Q.-R.); (S.O.-B.)
| | - Adrián Quevedo-Rodríguez
- Division of Rheumatology, Hospital Doctor Negrín, 35010 Las Palmas de Gran Canaria, Spain; (J.C.Q.-A.); (A.Q.-R.); (S.O.-B.)
| | - Alejandro Romo-Cordero
- Division of Internal Medicine, Hospital Universitario de Canarias, 38320 Tenerife, Spain; (A.R.-C.); (C.M.-G.)
| | - Soledad Ojeda-Bruno
- Division of Rheumatology, Hospital Doctor Negrín, 35010 Las Palmas de Gran Canaria, Spain; (J.C.Q.-A.); (A.Q.-R.); (S.O.-B.)
| | - Fuensanta Gómez-Bernal
- Division of Central Laboratory, Hospital Universitario de Canarias, 38320 Tenerife, Spain;
| | - Raquel López-Mejías
- Epidemiology, Genetics and Atherosclerosis Research Group on Systemic Inflammatory Diseases, Instituto de Investigación Sanitaria Marqués de Valdecilla (IDIVAL), 39011 Santander, Spain;
| | - Candelaria Martín-González
- Division of Internal Medicine, Hospital Universitario de Canarias, 38320 Tenerife, Spain; (A.R.-C.); (C.M.-G.)
- Department of Internal Medicine, Universidad de La Laguna (ULL), 38200 Tenerife, Spain
| | - Miguel Á. González-Gay
- Division of Rheumatology, IIS-Fundación Jiménez Díaz, 28040 Madrid, Spain
- Department of Medicine, University of Cantabria, 39005 Santander, Spain
- Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg 2000, South Africa
| | - Iván Ferraz-Amaro
- Division of Rheumatology, Hospital Universitario de Canarias, 38320 Tenerife, Spain;
- Department of Internal Medicine, Universidad de La Laguna (ULL), 38200 Tenerife, Spain
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Corredor-Orlandelli D, Arévalo-Romero A, Reyes C, Arango D. Massive Right Chylothorax Secondary to a Severe Systemic Lupus Erythematosus Flare With Secondary Evans Syndrome: A Case Report and Literature Review. CLINICAL MEDICINE INSIGHTS-CASE REPORTS 2023; 16:11795476231186735. [PMID: 37457318 PMCID: PMC10338652 DOI: 10.1177/11795476231186735] [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: 03/25/2023] [Accepted: 06/21/2023] [Indexed: 07/18/2023]
Abstract
This case report describes a 23-year-old male patient who presented with right chylothorax as the initial manifestation of a severe flare of systemic lupus erythematosus (SLE) and secondary Evans syndrome. Chylothorax and chylous ascites are rare features of SLE that can occur due to the accumulation of triglyceride-rich fluid in serous cavities. However, they have never been reported as the initial manifestation of a lupus flare. Evans syndrome is a rare disease characterized by autoimmune hemolytic anemia and immune thrombocytopenia, which can be secondary to SLE. The concomitant occurrence of both chylothorax and Evans syndrome in the setting of systemic lupus erythematosus has never been described, and the exact causative mechanisms of both entities are yet to be fully understood. In this report, we discuss our approach to this challenging case to broaden the understanding of the clinical manifestations of systemic lupus erythematosus. Our findings emphasize the importance of considering rare features of systemic lupus erythematosus and secondary diseases when evaluating patients with the disease.
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Affiliation(s)
- David Corredor-Orlandelli
- School of Medicine and Health Sciences, Universidad del Rosario, Bogotá, Colombia
- Medical Department, Fundación Clínica Abood-Shaio, Bogotá, Colombia
| | | | - Carlos Reyes
- Medical Department, Fundación Clínica Abood-Shaio, Bogotá, Colombia
- School of Medicine and Health Sciences, Universidad Militar, Bogotá, Colombia
| | - Dylan Arango
- Medical Department, Fundación Clínica Abood-Shaio, Bogotá, Colombia
- School of Medicine, Universidad del Bosque, Bogotá, Colombia
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11
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Shao D, Pena O, Sekulic M, Valdez Imbert R, Vegivinti CTR, Jim B. Secondary haemophagocytic lymphohistiocytosis in a patient with new-onset systemic lupus erythematosus: the challenges of timely diagnosis and successful treatment. BMJ Case Rep 2023; 16:e252938. [PMID: 37429644 PMCID: PMC10335589 DOI: 10.1136/bcr-2022-252938] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 07/12/2023] Open
Abstract
Haemophagocytic lymphohistiocytosis (HLH) is an immune-mediated disease driven by abnormal macrophage activation and regulatory cell dysfunction. HLH can be primary due to genetic mutations or secondary due to infection, malignancy or autoimmune conditions. We describe a woman in her early 30s who developed HLH while being treated for newly diagnosed systemic lupus erythematosus (SLE) complicated by lupus nephritis as well as concomitant cytomegalovirus (CMV) reactivation from a dormant infection. The trigger for this secondary form of HLH may have been either aggressive SLE and/or CMV reactivation. Despite prompt treatment with immunosuppressive therapies for SLE consisting of high-dose corticosteroids, mycophenolate mofetil, tacrolimus, etoposide for HLH and ganciclovir for CMV infection, the patient developed multiorgan failure and passed away. We demonstrate the difficulty in identifying a specific cause for secondary HLH when multiple conditions are present (SLE and CMV) and the fact that, despite aggressive treatment for both conditions, the mortality for HLH remains high.
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Affiliation(s)
- Daming Shao
- Medicine, Jacobi Medical Center, Bronx, New York, USA
| | - Oscar Pena
- Medicine, Jacobi Medical Center, Bronx, New York, USA
| | - Miroslav Sekulic
- Department of Pathology and Cell Biology, Columbia University Medical Center, New York, New York, USA
| | | | | | - Belinda Jim
- Medicine, Jacobi Medical Center, Bronx, New York, USA
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12
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Chen S, Zhang C, Chen D, Dong L, Chang T, Tang ZH. Advances in attractive therapeutic approach for macrophage activation syndrome in COVID-19. Front Immunol 2023; 14:1200289. [PMID: 37483597 PMCID: PMC10358730 DOI: 10.3389/fimmu.2023.1200289] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/04/2023] [Accepted: 06/12/2023] [Indexed: 07/25/2023] Open
Abstract
Nowadays, people have relaxed their vigilance against COVID-19 due to its declining infection numbers and attenuated virulence. However, COVID-19 still needs to be concern due to its emerging variants, the relaxation of restrictions as well as breakthrough infections. During the period of the COVID-19 infection, the imbalanced and hyper-responsive immune system plays a critical role in its pathogenesis. Macrophage Activation Syndrome (MAS) is a fatal complication of immune system disease, which is caused by the excessive activation and proliferation of macrophages and cytotoxic T cells (CTL). COVID-19-related hyperinflammation shares common clinical features with the above MAS symptoms, such as hypercytokinemia, hyperferritinemia, and coagulopathy. In MAS, immune exhaustion or defective anti-viral responses leads to the inadequate cytolytic capacity of CTL which contributes to prolonged interaction between CTL, APCs and macrophages. It is possible that the same process also occurred in COVID-19 patients, and further led to a cytokine storm confined to the lungs. It is associated with the poor prognosis of severe patients such as multiple organ failure and even death. The main difference of cytokine storm is that in COVID-19 pneumonia is mainly the specific damage of the lung, while in MAS is easy to develop into a systemic. The attractive therapeutic approach to prevent MAS in COVID-19 mainly includes antiviral, antibiotics, convalescent plasma (CP) therapy and hemadsorption, extensive immunosuppressive agents, and cytokine-targeted therapies. Here, we discuss the role of the therapeutic approaches mentioned above in the two diseases. And we found that the treatment effect of the same therapeutic approach is different.
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Affiliation(s)
- Shunyao Chen
- Department of Trauma Surgery, Emergency Surgery & Surgical Critical, Tongji Trauma Center, Wuhan, China
- Department of Emergency and Critical Care Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Cong Zhang
- Department of Trauma Surgery, Emergency Surgery & Surgical Critical, Tongji Trauma Center, Wuhan, China
- Department of Emergency and Critical Care Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Deng Chen
- Department of Trauma Surgery, Emergency Surgery & Surgical Critical, Tongji Trauma Center, Wuhan, China
- Department of Emergency and Critical Care Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Liming Dong
- Department of Trauma Surgery, Emergency Surgery & Surgical Critical, Tongji Trauma Center, Wuhan, China
- Department of Emergency and Critical Care Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Teding Chang
- Department of Trauma Surgery, Emergency Surgery & Surgical Critical, Tongji Trauma Center, Wuhan, China
- Department of Emergency and Critical Care Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Zhao-Hui Tang
- Department of Trauma Surgery, Emergency Surgery & Surgical Critical, Tongji Trauma Center, Wuhan, China
- Department of Emergency and Critical Care Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
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13
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Yao M, Huang X, Guo Y, Zhao JV, Liu Z. Disentangling the common genetic architecture and causality of rheumatoid arthritis and systemic lupus erythematosus with COVID-19 outcomes: Genome-wide cross trait analysis and bidirectional Mendelian randomization study. J Med Virol 2023; 95:e28570. [PMID: 36762574 DOI: 10.1002/jmv.28570] [Citation(s) in RCA: 14] [Impact Index Per Article: 7.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/15/2022] [Revised: 01/31/2023] [Accepted: 02/07/2023] [Indexed: 02/11/2023]
Abstract
Coronavirus Disease (COVID-19) may cause a dysregulation of the immune system and has complex relationships with multiple autoimmune diseases, including rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). However, little is known about their common genetic architecture. Using the latest data from COVID-19 host genetics consortium and consortia on RA and SLE, we conducted a genome-wide cross-trait analysis to examine the shared genetic etiology between COVID-19 and RA/SLE and evaluated their causal associations using bidirectional Mendelian randomization (MR). The cross-trait meta-analysis identified 23, 28, and 10 shared genetic loci for severe COVID-19, COVID-19 hospitalization, and SARS-CoV-2 infection with RA, and 14, 17, and 7 shared loci with SLE, respectively. Co-localization analysis identified five causal variants in TYK2, IKZF3, PSORS1C1, and COG6 for COVID-19 with RA, and four in CRHR1, FUT2, and NXPE3 for COVID-19 with SLE, involved in immune function, angiogenesis and coagulation. Bidirectional MR analysis suggested RA is associated with a higher risk of COVID-19 hospitalization, and COVID-19 is not related to RA or SLE. Our novel findings improved the understanding of the genetic etiology shared by COVID-19, RA and SLE, and suggested an increased risk of COVID-19 hospitalization in people with higher genetic liability to RA.
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Affiliation(s)
- Minhao Yao
- Department of Statistics and Actuarial Science, The University of Hong Kong, Hong Kong, Hong Kong, China
| | - Xin Huang
- School of Public Health, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong, Hong Kong, China
| | - Yunshan Guo
- Department of Statistics and Actuarial Science, The University of Hong Kong, Hong Kong, Hong Kong, China
| | - Jie V Zhao
- School of Public Health, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong, Hong Kong, China
| | - Zhonghua Liu
- Department of Biostatistics, Mailman School of Public Health, Columbia University, New York, New York, USA
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Liu PP, Shuai ZW, Lian L, Wang K. Systemic lupus erythematosus with multicentric reticulohistiocytosis: A case report. World J Clin Cases 2023; 11:456-463. [PMID: 36686350 PMCID: PMC9850977 DOI: 10.12998/wjcc.v11.i2.456] [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: 10/17/2022] [Revised: 12/08/2022] [Accepted: 12/23/2022] [Indexed: 01/12/2023] Open
Abstract
BACKGROUND Multicentric reticulohistiocytosis (MRH)/systemic lupus erythematosus (SLE) overlap syndrome is an uncommon disease in the clinic and is diagnosed through characteristic clinical manifestations, histopathology, and immunopathology. Here, we report the case of a 30-year-old woman with SLE who developed MRH.
CASE SUMMARY A 30-year-old woman with a history of polyarthritis for the past 12 years had multiple skin nodules on her body for 10 years, including the sacrococcygeal area, dorsum of the hands, interphalangeal joint of the feet and sternoclavicular joint. The histopathology of a biopsy of the distal interphalangeal joint of the hands revealed granulomatous inflammation, fibrous hyperplasia with ground-glass degeneration, inflammatory cell exudation and focal necrosis. The immunohistochemical stains showed positive staining for CD68 and negative staining for S100 and acid-fast staining. The patient was diagnosed with SLE with MRH. Her symptoms were improved after a combined treatment of prednisone, hydroxychloroquine and cyclophosphamide.
CONCLUSION MRH/SLE overlap syndrome is difficult to diagnose and treat. Cyclophosphamide may be an alternative choice for the treatment of MRH.
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Affiliation(s)
- Ping-Ping Liu
- Department of Rheumatology and Immunology, Arthritis Research Institute, The First Affiliated Hospital of Anhui Medical University, Hefei 230000, Anhui Province, China
| | - Zong-Wen Shuai
- Department of Rheumatology and Immunology, Arthritis Research Institute, The First Affiliated Hospital of Anhui Medical University, Hefei 230000, Anhui Province, China
| | - Li Lian
- Department of Rheumatology and Immunology, Arthritis Research Institute, The First Affiliated Hospital of Anhui Medical University, Hefei 230000, Anhui Province, China
| | - Kang Wang
- Department of Rheumatology and Immunology, Arthritis Research Institute, The First Affiliated Hospital of Anhui Medical University, Hefei 230000, Anhui Province, China
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15
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Tharwat S, Hamdy F, Abdelzaher MG, Bakr L, El Hassany A, Abdelsattar M, Eldesoky RT, Ibrahim EM. Life threatening macrophage activation syndrome as the initial presentation of systemic lupus erythematosus: A case report and review of the literature. THE EGYPTIAN RHEUMATOLOGIST 2023. [DOI: 10.1016/j.ejr.2022.10.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
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16
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Dey S, Roongta R, Mondal S, Haldar S, Sircar G, Ghosh B, Ghosh A. Recurrent macrophage activation syndrome as the initial manifestation of paediatric systemic lupus erythematosus. Lupus 2022; 31:1132-1137. [PMID: 35713230 DOI: 10.1177/09612033221108852] [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: 11/15/2022]
Abstract
Introduction: Macrophage Activation Syndrome (MAS) is a rare but potentially fatal complication in rheumatic diseases. Here, we report the case of a 14-year-old girl with MAS as the primary manifestation of Systemic Lupus Erythematosus (SLE). She had three episodes of MAS during the course of her treatment. This case is unique as recurrent MAS in pediatric SLE is rare.Methods: Demographic, clinical, laboratory features and outcomes of our patient was noted. We also reviewed the two reported cases of recurrent MAS in pediatric SLE. Literature review was performed on PubMed search forum. Search items included Macrophage activation syndrome, pediatric systemic lupus erythematosus, recurrent MAS.Conclusion: The diagnosis and management of MAS are challenging as it can simulate an infectious complication or can be the exacerbation of the underlying disease. Early detection and prompt treatment can reduce morbidity in these patients.
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Affiliation(s)
- Sonali Dey
- Department of Clinical Immunology and Rheumatology, 30164Institute of Postgraduate Medical Education and Research, Kolkata, India
| | - Rashmi Roongta
- Department of Clinical Immunology and Rheumatology, 30164Institute of Postgraduate Medical Education and Research, Kolkata, India
| | - Sumantro Mondal
- Department of Clinical Immunology and Rheumatology, 30164Institute of Postgraduate Medical Education and Research, Kolkata, India
| | - Subhankar Haldar
- Department of Clinical Immunology and Rheumatology, 30164Institute of Postgraduate Medical Education and Research, Kolkata, India
| | - Geetabali Sircar
- Department of Clinical Immunology and Rheumatology, 30164Institute of Postgraduate Medical Education and Research, Kolkata, India
| | - Biswadip Ghosh
- Department of Clinical Immunology and Rheumatology, 30164Institute of Postgraduate Medical Education and Research, Kolkata, India
| | - Alakendu Ghosh
- Department of Clinical Immunology and Rheumatology, 30164Institute of Postgraduate Medical Education and Research, Kolkata, India
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Poursac N, Odriozola I, Truchetet ME. Strategy and Challenges of Paraclinical Examinations in Adult-Onset Still’s Disease. J Clin Med 2022; 11:jcm11082232. [PMID: 35456325 PMCID: PMC9027491 DOI: 10.3390/jcm11082232] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/10/2022] [Revised: 04/13/2022] [Accepted: 04/13/2022] [Indexed: 02/01/2023] Open
Abstract
Adult-onset Still’s disease is a complex autoinflammatory disease with a multifactorial etiology. Its presentation is less stereotypical than that of a monogenic autoinflammatory disease and is actually relatively common with few specific signs. To avoid under- or over-prescription of complementary examinations, it is useful to advance in a structured manner, taking into consideration the actual added value of each supplemental examination. In this review, we detail the different complementary tests used in adult Still’s disease. We consider them from three different angles: positive diagnostic approach, the differential diagnosis, and the screening for complications of the disease. After discussing the various tests at our disposal, we look at the classical diagnostic strategy in order to propose a structured algorithm that can be used in clinical practice. We conclude with the prospects of new complementary examinations, which could in the future modify the management of patients.
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Affiliation(s)
- Nicolas Poursac
- Department of Rheumatology, FHU ACRONIM, University Hospital of Bordeaux, 33000 Bordeaux, France; (N.P.); (I.O.)
| | - Itsaso Odriozola
- Department of Rheumatology, FHU ACRONIM, University Hospital of Bordeaux, 33000 Bordeaux, France; (N.P.); (I.O.)
| | - Marie-Elise Truchetet
- Department of Rheumatology, FHU ACRONIM, University Hospital of Bordeaux, 33000 Bordeaux, France; (N.P.); (I.O.)
- Immunology Laboratory, ImmunoConcept, UMR CNRS 5164, University Hospital of Bordeaux, 33000 Bordeaux, France
- Correspondence:
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Celiac Disease in Juvenile Idiopathic Arthritis and Other Pediatric Rheumatic Disorders. J Clin Med 2022; 11:jcm11041089. [PMID: 35207358 PMCID: PMC8878661 DOI: 10.3390/jcm11041089] [Citation(s) in RCA: 9] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/21/2022] [Revised: 02/10/2022] [Accepted: 02/16/2022] [Indexed: 02/08/2023] Open
Abstract
Celiac Disease (CD) is an immune-mediated and gluten-related disorder whose prevalence is higher in children affected with other autoimmune disorders, including diabetes mellitus type 1, autoimmune thyroiditis, and others. As regards Juvenile Idiopathic Arthritis (JIA) and other pediatric rheumatic disorders, there is no clear recommendation for CD serological screening. In this review, we analyze all the available clinical studies investigating CD among children with JIA (and other rheumatic diseases), in order to provide objective data to better understand the necessity of CD serological screening during the follow-up. Based on the present literature review and analysis, >2.5% patients with JIA were diagnosed with CD; however, the CD prevalence in JIA patients may be even higher (>3–3.5%) due to several study limitations that could have underestimated CD diagnosis to a variable extent. Therefore, serological screening for CD in children affected with JIA could be recommended due to the increased CD prevalence in these patients (compared to the general pediatric population), and because these JIA patients diagnosed with CD were mostly asymptomatic. However, further research is needed to establish a cost-effective approach in terms of CD screening frequency and modalities during the follow-up for JIA patients. Conversely, at the moment, there is no evidence supporting a periodical CD screening in children affected with other rheumatic diseases (including pediatric systemic lupus erythematosus, juvenile dermatomyositis, and systemic sclerosis).
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