|
| Autoantibody | Autoantigen | Prevalence | Response to treatment | Significance |
|
| Anti-TIF1-γ (anti-p155) | Transcription intermediary factor 1-γ | 32% | This subset of patients more often receive treatment with a biologic drugs and/or intravenous cyclophosphamide | Severe cutaneous disease: lipodystrophy, skin ulceration and edema |
| Ani-Mi-2 | Nucleosome remodeling deacetyalse complex (NuRD) | 4-10% | Respond well to conventional therapy | Greater muscle weakness, dysphagia, and edema |
| Anti-MDA5 (anti-CADM-140) | Melanoma differentiation-associated protein 5 | 7-33% | Frequently requires intensive immunosuppressive therapy combining several immunosuppressants | Rapidly progressive ILD, higher IL-18, IL-6, and ferritin levels |
| Anti-NXP2 | Nuclear matrix protein 2b | 15-23% | Required more aggressive treatment with a lower remission rate during the follow-up period | Severe muscle weakness, joint contractures, intestinal vasculitis, polyarthritis, calcinosis |
| Anti-SAE | Small ubiquitin-like modifier activating enzyme | 1% | Respond well to conventional therapy | Amyopathic dermatomyositis |
| Anti-SRP | Signal recognition peptide (SRP) | 1.6% | Poorly responsive to standard treatment, however, with satisfying response to aggressive treatment with a combination of rituximab, cyclophosphamide and IVIGs, followed by maintenance methotrexate and intensive daily physical therapy | Immune-mediated necrotizing myositis, severe muscle disease, cardiac involvement |
| Anti-HMGCR | HMG-CoA reductase (3-hydroxy-3-methyl-glutaryl-coenzyme A reductase (HMGCR) | 1% | Poorly responsive to standard treatment, only partial responses to multiple immunosuppressive medications | Immune-mediated necrotizing myositis, worse disease course |
Anti-ARS:
(i) Anti-Jo-1
(ii) Anti-PL-7
(iii) Anti-PL-12
(iv) Anti-EJ
(v) Anti-OJ
(vi) Anti-KS
(vii) Anti-Zo
(viii) Anti-YRS/Ha | Aminoacyl-tRNA synthetases (ARS):
(i) Histidyl-tRNA synthetase
(ii) Treonyl-tRNA synthetase
(iii) Alanyl-tRNA synthetase
(iv) Glycyl-tRNA synthetase
(v) Isoleucyl-tRNA synthetase
(vi) Asparaginyl-tRNA synthetase
(vii) Phenylalanine-tRNA synthetase
(viii) Tyrosyl-tRNA synthetase | <5% | Glucocorticoids are the empirical first-line therapy; however, additional immunosuppressive agents are often necessary | Anti-synthetase syndrome; myositis, ILD, fever, Raynaud’s phenomenon, arthritis, and mechanic’s hands. |
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