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Clinical features, muscle biopsy scores, myositis specific antibody profiles and outcome in juvenile dermatomyositis

Seminars in Arthritis and Rheumatism Dec 25, 2020

Sag E, Demir S, Bilginer Y, et al. - Fifty-eight patients (35F, 23 M) from a tertiary referral center in the last two decades were involved to analyze clinical characteristics, paraclinical examinations, myositis specific antibodies (MSAs), treatment response and long-term outcome in a juvenile dermatomyositis (JDM) cohort. The key diagnostic elements were dermatological manifestations and muscle weakness. The diagnosis was compatible with elevated serum creatine kinase levels, electromyography, muscle MRI, and muscle biopsy. Calcinosis, associated with disease onset ≤ 6 years, higher muscle biopsy scores and MDA5 positivity, was the most common long-term complication. Integration of clinical features with the results of laboratory and biopsy will help predict prognosis and guide treatment in JDM. Age, MDA5 autoantibodies, and muscle biopsy scores were correlated with calcinosis in the cohort.

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