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Table 1 Myositis-specific autoantibodies, target autoantigens, and clinical associations

From: Novel autoantibodies and clinical phenotypes in adult and juvenile myositis

    Frequency, percentage
Autoantibody Target autoantigen Clinical associations Adults JDM
Anti-ARS Amino-acyl-tRNA synthetase Anti-synthetase syndrome Overall: 30-40 Overall: 1-3
- Jo-1 - Histidyl Myositis Jo-1: 15-20  
- PL7 - Theronyl Interstitial lung disease PL7: <5  
- PL12 - Alanyl Raynaud phenomenon PL12: <5  
- OJ - Isoleucyl Arthritis OJ: <5  
- EJ - Glycyl Mechanic's hands EJ: <5  
- KS - Asparaginyl Fever KS: <5  
- Ha - Tyrosyl   Ha: <1  
- Zo - Phenylalanyl   Zo: <1  
Anti-Mi-2 Nucleosome remodeling deacetyalse complex (NuRD) DM < 10 4-10
Anti-p155/140 Transcriptional intermediary factor 1 gamma (TIF1-γ) JDM: DM and ulceration 13-21 22-29
   Adults: DM and malignancy   
Anti-p140 Nuclear matrix protein 2 (NXP2) JDM: DM and calcinosis < 5 23
   Adults: DM and ILD   
Anti-SAE Small ubiquitin-like modifier activating enzyme (SAE) DM < 5 < 1
Anti-CADM-140 Melanoma differentiation-associated gene 5 (MDA5) CADM and ILD 50-73 CADM (not in Caucasians) Not known
Anti-SRP Signal recognition particle (SRP) Necrotizing myopathy 5-10 < 3
Anti-200/100 Unknown 100- and 200-kDa proteins Necrotizing myopathy < 10 necrotizing myopathy Not known
  1. ARS, amino-acyl-tRNA synthetase; CADM, clinically amyopathic dermatomyositis; DM, dermatomyositis; ILD, interstitial lung disease; JDM, juvenile dermatomyositis.