Myositis-Specific and -Associated Autoantibodies
Autoantibodies | Clinical Features |
|---|---|
Antisynthetase syndrome* | |
Anti-Jo-1 | Myositis† Interstitial lung disease |
Anti-PL-7 | Interstitial lung disease† Myositis |
Anti-PL-12 | Interstitial lung disease† Myositis |
Anti-EJ | Interstitial lung disease Myositis |
Anti-OJ | Interstitial lung disease Myositis |
Anti-KS | Interstitial lung disease† Myositis |
Anti-Zo | Interstitial lung disease Myositis |
Anti-Ha | Interstitial lung disease Myositis |
Dermatomyositis | |
Anti-Mi-2 | Severe skin disease Responds well to treatment |
Anti-MDA5 | Cardiopulmonary syndrome, severe interstitial lung disease Amyopathic dermatomyositis, palmar papules, skin ulcers |
Anti-TIF1g | Increased cancer risk |
Anti-NXP-2 | Subcutaneous calcinosis Increased cancer risk |
Immune-mediated necrotizing myopathy | |
Anti-SRP | Severe, difficult to treat |
Anti-HMGCR | Immune-mediated statin-associated myopathy (can rarely be present without previous exposure to prescription statins); may persist after stopping statin therapy |
Inclusion body myositis | |
Cytosolic 5'-nucleotidase 1A | Inclusion body myositis |
Overlap syndrome | |
anti-PM-Scl | Myositis Systemic sclerosis Interstitial lung disease Mechanic's hands, calcinosis, Raynaud phenomenon |
anti-Ku | Myositis Systemic sclerosis (particularly limited cutaneous systemic sclerosis) Interstitial lung disease Arthritis, esophageal involvement, Raynaud phenomenon |
anti-U3-RNP | Myositis Systemic sclerosis (particularly diffuse cutaneous systemic sclerosis) |
* All of these autoantibodies can be present in patients with complete or incomplete forms of antisynthetase syndrome. Antisynthetase syndrome features include fever, nonerosive arthritis, interstitial lung disease, hyperkeratosis of the radial aspect of the digits (mechanic's hands), and Raynaud phenomenon. The rash of dermatomyositis may or may not be present. Patients with antisynthetase syndrome antibodies and muscle disease may have findings of dermatomyositis or polymyositis. | |
† This feature is the more prominent one. | |
Data from Mammen A: Autoimmune muscle disease. Handb Clin Neurol. 2016;133:467–484. doi:10.1016/B978-0-444-63432-0.00025-6 and from Fredi M, Cavazzana I, Franceschini F. The clinico-serological spectrum of overlap myositis. Curr Opin Rheumatol. 2018;30(6):637-643. doi:10.1097/BOR.0000000000000536. | |