Autoantibodies Clinical features
Anti-ARS (antisynthetase) AS syndrome with moderate-to-serve muscle weakness with high muscle enzyme levels, RP, mechanic’s hands, fever, arthritis, and ILD
Anti-Jo-1 Chronic continuous disease course, with clinical symptoms for >two years after diagnosis; mean five-year survival rate = 65%, usually due to ILD; AS syndrome features
Anti-PL-7 AS syndrome with higher frequency of ILD
Anti-PL-12 AS syndrome with higher frequency of ILD
Anti-EJ Dermatomyositis and ILD
Anti-OJ Myositis and ILD
Anti-KS ILD with less myositis
Anti-Ha NA
Anti-Zo Myositis and ILD
Anti-SRP Acute onset NM with severe weakness, high CK, cardiac involvement; refractory to treatment
Anti-Mi-2 Adult DM and JDM with hallmark cutaneous disease, milder myositis with good response to treatment
Anti-TIF1-y (anti-p155/140) CAM in adult DM; severe cutaneous disease in adult DM and JDM
Anti-SAE Adult DM; may present with CADM first
Anti-MDA5 (anti-CADM140) CADM; rapidly progressive ILD
Anti-NXP-2 Predominantly JDM with subcutaneous edema, calcinosis, and servere muscle disease with contractures; increased risk of cancer in some adult DM studies
Anti-HMGCR NM; associated with statin use in adults; severe proximal muscle weakness; partially responsive to immunosuppressive medications; better response to IVIg
Anti-cN-1A (Mup44, NT5c1A) IBM; higher mortality risk

ARS=aminoacyl-tRNA synthetase; AS syndrome=antisynthetase syndrome; RP=Raynaud’s phenomenon; ILD=interstital lung disease; SRP=signal recognition particle; T1F1-y=transcriptional intermediary factory 1-gamma; NXP-2=nuclear matrix protein-2; SAE=small-ubiquitin-like modifier activating enzyme; MDA5=melanoma-differentiation associated gene 5; CAM=cancer-associated myositis; CADM-clinically amyopathic DM; NA-not applicable/no data.