Research progress of juvenile dermatomyositis with anti-melanoma differentiation-associated gene 5 antibody / 中华实用儿科临床杂志

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.