Wedderburn and Rider 2009 Table 1.
Myositis specific autoantibodies(MSAs) and Myositis associated autoantibodies (MAAs) in patients with JDM
| Autoantibody | Autoantigen target | Frequency in Caucasian patients * % |
Clinical features and associations in JDM |
|---|---|---|---|
| MSAs | |||
| Anti-ARS: | Aminoacyl-tRNA synthetases*: | 1-5 | Typically associated with those with moderate to severe weakness, may have arthritis, mechanics hands, Raynaud’s, fevers, interstitial lung disease |
| Anti-Jo-1 | Histidyl-tRNA synthetase | 2-5 | |
| Anti-PL-12 | Alanyl-tRNA synthetase | 1-3 | |
| Anti-PL-7 | Threonyl-tRNA synthetase | <1 | |
| Anti-EJ | Glycyl-tRNA synthetase | <1 | |
| Anti-OJ | Isoleucyl-tRNA synthetase | <1 | |
| Anti-KS | Asparagynyl-tNRA synthearin)tase | NA | |
| Anti-Ha | Tyrosyl-tRNA synthetase | NA | |
| Anti-Zo | Phenylalanyl-tRNA synthetase | NA | |
| Other MSAs | |||
| Anti-Mi-2 | DNA Helicase | ~5 | Classical cutaneous JDM |
| Anti-SRP | Signal recognition particle | 1-3 | Associated with severe, refractory polymyositis |
|
Recently-Identified Myositis Autoantibodies: |
|||
| Anti-p155/140 or Anti-p155 |
Transcriptional intermediary factor (TIF)-1 gamma protein |
23-29 | More severe cutaneous involvement, generalized lipodystrophy |
| Anti-p140 (MJ) | Thought to be the MJ autoantigen, nuclear matrix protein NXP2 |
13-23 | Associated with calcinosis, contractures |
| MAAs | |||
| Anti-U1-RNP | U1 ribonucleoprotein (snRNP) | ~6 | Associated with sclerodermatous overlap features |
| Anti-U3-RNP | U3 ribonucleoprotein (fibrillarin) | ~1 | Associated with sclerodermatous overlap features |
| Anti-PM-Scl | Nucleolar multi-protein complex | 5-7 | Associated with sclerodermatous overlap features |
| Anti-Ro | 52 or 60Kd ribonuceloproteins (hYRNA) | ~2 | |
| Anti-La | Ribonuceloprotein | ~1 | |
| Anti-Ku | p70/p80 heterodimer, DNA-associated proteins | ~1 | |
| Anti-Topo | DNA Topoisomerase 1 | 1 | |
*
Frequencies quoted are from data in Caucasian patients; however frequencies vary considerably in different ethnicities. Notably certain autoantibodies which are higher in African American patients include anti-Jo1, anti-SRP, anti-Ro and anti-URNP [24]. NA- not available asdata not yet reported in cohorts of JDM patients.