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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.