Autoantibodies in Autoimmune Myositis

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

Amyopathic dermatomyositis, palmar papules

Anti-TIF1g

Increased cancer risk

Anti-NXP-2

Subcutaneous calcinosis

Increased cancer risk

Necrotizing immune-mediated myopathy

Anti-SRP

Severe, difficult to treat

Anti-HMGCR

Immune-mediated statin-associated myopathy (can rarely be present without previous exposure to prescription statins)

Inclusion body myositis

Cytosolic 5'-nucleotidase 1A

Inclusion body myositis

* 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 syndrome. 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.

Modified from Mammen A: Autoimmune Muscle Disease. In Handbook of Clinical Neurology, edited by SJ Pittock and A Vincent. Elsevier BV, 2016, pp. 467–484. doi:10.1016/B978-0-444-63432-0.00025-6