Twelve years experience of juvenile dermatomyositis in North India

Abstract

The aim of this study was to evaluate outcome in children with juvenile dermatomyositis (JDMS) at a tertiary care center in North India and have a long term follow-up. Medical records of children with JDMS managed at a tertiary care hospital were reviewed during a 13-year period to determine (1) interval between onset of symptoms and diagnosis, (2) treatment modalities used and (3) long term functional outcome. Thirty three patients diagnosed with JDMS met the inclusion criteria. Mean age at diagnosis was 8.7 +/- 3.3 years. Mean duration of disease prior to treatment was 1.18 years (range 1 month-5 years). The total follow-up period was 136.7 patient-years. Immunosuppressive therapy was given in 31/33 and a distinct monocyclic course was seen in 72.7% cases. Lipodystrophy was seen in 10/33 (30.3%), calcinosis in 7/33 (27.3%), cutaneous ulcers in 6/33 (18.2%), dysphagia in 5/33 (15.2%), and contractures in 4/33 (12.1%) cases. A steady and sustained response was seen in patients who had received "adequate" doses of steroids at the time of initiation of treatment. Methotrexate, hydroxychloroquine, azathioprine and intravenous immunoglobulin were used in patients with poor response to corticotherapy. There were two deaths in our series. Stepwise, aggressive treatment directed at achieving rapid and complete control of muscle inflammation is highly successful in minimizing the long-range sequelae of JDMS. Our patients seem to have a different clinical profile on follow-up as compared to series published from the West.