Research progress of juvenile dermatomyositis with anti-melanoma differentiation-associated gene 5 antibody
10.3760/cma.j.cn101070-20220301-00214
- VernacularTitle:抗黑色素瘤分化相关基因5抗体阳性幼年皮肌炎的研究进展
- Author:
Junmei ZHANG
1
;
Caifeng LI
Author Information
1. 国家儿童医学中心，首都医科大学附属北京儿童医院风湿科，北京 100045
- Keywords:
Juvenile dermatomyositis;
Melanoma differentiation associated gene 5;
Myositis-specific autoantibodies
- From:
Chinese Journal of Applied Clinical Pediatrics
2023;38(3):233-237
- CountryChina
- Language:Chinese
-
Abstract:
Juvenile dermatomyositis (JDM) is the most common subtype of juvenile idiopathic inflammatory myopathy (JIIM), characterized by non-suppurative inflammation of skin and muscle.JDM frequently involves important organs such as lungs.JDM with anti-melanoma differentiation-associated gene (MDA) 5 antibody has unique clinical characteristics, mainly including skin mucosal ulcer, palm papule, hair loss and arthritis.Interstitial lung disease (ILD) is its most serious complication.The levels of serum ferritin, Krebs von den Lungen-6 and interleukin-18 can be used as important indicators of disease activity and prognosis.Glucocorticoids combined with immunosuppressants are the basic treatment for the disease.Immunosuppressants include calcineurin inhibitors (Cyclosporine A and Tacrolimus), Cyclophosphamide, Azathioprine, Mycophenolate Mofetil, etc.Refractory patients can also be treated with Rituximab, Janus kinase inhibitor and human immunoglobulin.Early active treatment of JDM with anti-MDA 5 antibody can alleviate the symptoms, reverse organ damage and improve the long-term prognosis.
