| 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.