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Table 3 Rare autoantibodies in myositis and reported clinical associations.

From: Personalized medicine in rheumatology: the paradigm of serum autoantibodies

Autoantibody Prevalence (%) Clinical associations References
Anti-ARS 1–30 Anti-synthetase syndrome [69, 95]
Anti-Mi2 10 DM with typical skin lesions, mild disease [69, 70, 95]
Anti-TIF1γ 10–15 Severe DM, malignancies [70, 95]
Anti-NPX2 1–5 Severe DM, severe skin disease, malignancies [69, 70, 95]
Anti-MDA5 15–20 CADM, ILD, severe skin manifestations, poor prognosis [69, 95, 96]
Anti-SAE 1 Amyopathic DM [95]
Anti-SRP 5 Necrotizing myopathy [70]
Anti-HMGCR 6 Necrotizing myopathy, proximal muscle weakness, elevated CK levels, prior statin use [70, 95, 97]
  1. Anti-ARS anti-aminoacyl tRNA synthetases (Jo1, PL-7, PL-12, EJ, OJ, KS), DM dermatomyositis, CADM clinically amyopathic dermatomyositis, ILD interstitial lung disease, CK creatine kinase