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This research was supported in part by the Intramural Research Program of the National Institute of Arthritis and Musculoskeletal and Skin Diseases of the NIH. S.K.D., L.C.S., and the Myositis Research Database are supported by the Huayi and Siuling Zhang Discovery Fund. I.P.-F.'s research is supported by a Fellowship from the Myositis Association. J.J.P. is supported by the Doris Duke Early Investigator Award. E.T. is supported by grants from the Rheumatology Research Foundation and the Jerome L. Greene Foundation.

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Muscular and extramuscular clinical features of patients with anti-PM/Scl autoantibodies

Publicat a:Neurology. 90 (23): E2068-E2076 - 2018-06-05 90(23), DOI: 10.1212/WNL.0000000000005638

Autors: De Lorenzo, Rebecca; Pinal-Fernandez, Iago; Huang, Wilson; Albayda, Jemima; Tiniakou, Eleni; Johnson, Cheilonda; Milisenda, Jose C; Casal-Dominguez, Maria; Corse, Andrea M; Danoff, Sonye K; Christopher-Stine, Lisa; Paik, Julie J; Mammen, Andrew L

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To define the clinical features of myositis patients with anti-PM/Scl-75 and/or anti-PM/Scl-100 autoantibodies at disease onset and during the course of disease and compare them to patients with other forms of myositis.In this longitudinal cohort study, the prevalence and severity of clinical features at disease onset and during follow-up were compared between anti-PM/Scl-positive patients and those with the antisynthetase syndrome (AS), dermatomyositis (DM), and immune-mediated necrotizing myopathy (IMNM).Forty-one anti-PM/Scl-positive, 132 AS, 178 DM, and 135 IMNM patients were included. Although muscle weakness was a presenting feature in just 37% of anti-PM/Scl-positive patients, 93% eventually developed weakness. Unlike the other groups, anti-PM-Scl-positive patients had more severe weakness in arm abductors than hip flexors. Interstitial lung disease was a presenting feature in just 10% of anti-PM/Scl-positive patients, but occurred in 61% during follow-up; fewer patients with DM (13%, p < 0.001) and IMNM (6%, p < 0.001) and more patients with AS (80%, p < 0.05) developed interstitial lung disease during the course of disease. Mechanic's hands (80%), Raynaud syndrome (78%), sclerodactyly (66%), telangiectasias (66%), esophageal reflux disease (61%), subcutaneous edema (46%), puffy hands (39%), and calcinosis (39%) occurred more frequently in anti-PM/Scl-positive patients than in the other groups. Although 30% of anti-PM/Scl-positive patients met criteria for systemic sclerosis, less than 5% had renal crisis or finger ulcerations. No differences were found between patients with only anti-PM/Scl-100 or only anti-PM/Scl-75 autoantibodies.Unlike patients with DM, AS, or IMNM, anti-PM/Scl-positive patients have weaker arm abductors than hip flexors. Anti-PM/Scl-positive patients also have the most extensive extramuscular features.© 2018 American Academy of Neurology.

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