JAKi for Dermatomyositis

Dermatomyositis (DM) is a systemic autoimmune rheumatic disease which can affect multi-organs, such as skin, muscle and lung. Five myositis-specific autoantibodies, i.e., TIF1r, MDA5, NXP2, Mi2 and SAE, are sero-markers of diagnostic value. The treatments for DM included glucocorticoids, csDMARDs, IVIg and biologics, e.g., rituximab. Type I IFN pathway is considered to be crucial in the pathogenesis of DM and the evidence of JAK inhibitors as treatment option in DM is accumulating. Herein, four clinical scenarios of DM, i.e., MDA5+DM-ILD, TIF1r+ cancer-associated DM, NXP2+ adult DM with GI involvement, and MSA negative DM, are presented to reveal the trials and tribulations to tackle this very difficult disease.