Progress in diagnosis and treatment of anti-MDA5 antibody-related dermatomyositis / 中华检验医学杂志

ABSTRACT

Dermatomyositis (DM) with positive anti-melanoma differentiation-associated gene 5 (MDA5) antibodies (MDA5+DM) is a kind of occasional and rare autoimmune disease. Due to the fact that MDA5+DM patients are prone to suffer from the rapid progressive interstitial lung disease (RP-ILD), and the mortality rate is extremely high (all-cause mortality at 6 months is almost 50%). In addition to lung disease, patients with MDA5+DM also suffering from the skin and muscle symptoms. The biomarkers represented by the anti-MDA5 antibody titer, ferritin, KL-6 level and CD4 +/CXCR4 +T cell percentage are considered to relate with MDA5+DM-ILD′s severity, activity evaluation, therapeutic effect monitoring, and prognosis prediction. The current therapeutic strategies for the disease is mainly combined with immunosuppression. This work systemly summarizes the diagnosis and treatment progress of anti-mda5 antibody-related dermatomyositis, which not only contributes to the research work of related disciplines, but also provides reference for clinical diagnosis and treatment.