Abstract
Idiopathic inflammatory myopathies (IIM) are a group of refractory heterogeneous autoimmune diseases with polymyositis (PM) and skeletal myositis infiltration with multiple organ involvement. Derm-atomyositis (DM) is a common clinical subtype of IIM. IIM autoantibodies include myositis specific au-toant-ibodies (MSAs) and myositis associated autoantibodies (MAAs). Recent studies have found anti-synthetase antibody profiles (including anti-Jo-1, anti-PL-7 and anti-PL-12 antibodies), anti-Mi-2 antibodies, signal recognition particle (SRP) antibodies, and anti-synthesis hydroxy-3-methyl-glutaryl-coa reductase (H-MGCR) antibody, melanoma differentiation associa-ted gene 5 (MDA5) antibody, nuclear matrix protein 2 (NXP2) antibodies and transcriptional intermediation factor 1γ (TIF1γ) antibodies are strongly associated with IMM or clinical features of different clinical subtypes, early diagnosis of IIM, suggesting organ involvement and prognosis Aspects are of paramount importance. This article is to understand the clinical features of anti-synthetase syndrome by studying a patient with JO-positive of dermatomyositis.