Performance of interferon-gamma levels may lead to earlier diagnosing macrophage activation syndrome complicating systemic juvenile idiopathic arthritis

Abstract Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of rheumatic diseases, predominantly in systemic juvenile idiopathic arthritis (SJIA), and is considered as an autoinflammatory disease. Specific cytokine profiles could play a pivotal role in this inflammatory...

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Main Authors: Meiping Lu, Liping Teng, Yiping Xu, Xuefeng Xu
Format: Article
Language:English
Published: BMC 2023-10-01
Series:Pediatric Rheumatology Online Journal
Subjects:
Online Access:https://doi.org/10.1186/s12969-023-00907-7
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author Meiping Lu
Liping Teng
Yiping Xu
Xuefeng Xu
author_facet Meiping Lu
Liping Teng
Yiping Xu
Xuefeng Xu
author_sort Meiping Lu
collection DOAJ
description Abstract Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of rheumatic diseases, predominantly in systemic juvenile idiopathic arthritis (SJIA), and is considered as an autoinflammatory disease. Specific cytokine profiles could play a pivotal role in this inflammatory response. Gram-negative bacteremia, bacterial pneumonia, Kawasaki disease, and active SJIA exhibited similar cytokine profiles with elevated interleukin-6 (IL-6) and/or IL-10, further suggesting a correlation between them. Only when JIA is complicated by MAS can increased interferon-γ (IFN-γ) levels be observed. Therefore, increased serum IFN-γ levels could contribute to early diagnosing MAS in patients with SJIA in combination with other variables such as serum ferritin. A prospective multi-center study will be performed to further confirm the role of IFN-γ in the early recognition of MAS in SJIA.
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spelling doaj.art-34853b8934e14eb48061fed740e8fb482023-11-19T12:39:59ZengBMCPediatric Rheumatology Online Journal1546-00962023-10-012111310.1186/s12969-023-00907-7Performance of interferon-gamma levels may lead to earlier diagnosing macrophage activation syndrome complicating systemic juvenile idiopathic arthritisMeiping Lu0Liping Teng1Yiping Xu2Xuefeng Xu3Department of Rheumatology Immunology & Allergy, Children’s Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child HealthDepartment of Rheumatology Immunology & Allergy, Children’s Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child HealthDepartment of Rheumatology Immunology & Allergy, Children’s Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child HealthDepartment of Rheumatology Immunology & Allergy, Children’s Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child HealthAbstract Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of rheumatic diseases, predominantly in systemic juvenile idiopathic arthritis (SJIA), and is considered as an autoinflammatory disease. Specific cytokine profiles could play a pivotal role in this inflammatory response. Gram-negative bacteremia, bacterial pneumonia, Kawasaki disease, and active SJIA exhibited similar cytokine profiles with elevated interleukin-6 (IL-6) and/or IL-10, further suggesting a correlation between them. Only when JIA is complicated by MAS can increased interferon-γ (IFN-γ) levels be observed. Therefore, increased serum IFN-γ levels could contribute to early diagnosing MAS in patients with SJIA in combination with other variables such as serum ferritin. A prospective multi-center study will be performed to further confirm the role of IFN-γ in the early recognition of MAS in SJIA.https://doi.org/10.1186/s12969-023-00907-7Autoinflammatory DiseaseCytokineDiagnosisMacrophage activation syndromeSystemic juvenile idiopathic arthritis
spellingShingle Meiping Lu
Liping Teng
Yiping Xu
Xuefeng Xu
Performance of interferon-gamma levels may lead to earlier diagnosing macrophage activation syndrome complicating systemic juvenile idiopathic arthritis
Pediatric Rheumatology Online Journal
Autoinflammatory Disease
Cytokine
Diagnosis
Macrophage activation syndrome
Systemic juvenile idiopathic arthritis
title Performance of interferon-gamma levels may lead to earlier diagnosing macrophage activation syndrome complicating systemic juvenile idiopathic arthritis
title_full Performance of interferon-gamma levels may lead to earlier diagnosing macrophage activation syndrome complicating systemic juvenile idiopathic arthritis
title_fullStr Performance of interferon-gamma levels may lead to earlier diagnosing macrophage activation syndrome complicating systemic juvenile idiopathic arthritis
title_full_unstemmed Performance of interferon-gamma levels may lead to earlier diagnosing macrophage activation syndrome complicating systemic juvenile idiopathic arthritis
title_short Performance of interferon-gamma levels may lead to earlier diagnosing macrophage activation syndrome complicating systemic juvenile idiopathic arthritis
title_sort performance of interferon gamma levels may lead to earlier diagnosing macrophage activation syndrome complicating systemic juvenile idiopathic arthritis
topic Autoinflammatory Disease
Cytokine
Diagnosis
Macrophage activation syndrome
Systemic juvenile idiopathic arthritis
url https://doi.org/10.1186/s12969-023-00907-7
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