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.

Profile of Pediatric Idiopathic Inflammatory Myopathies from a Tertiary Care Center of Eastern India

“…Most of the earlier Indian studies have shown a male predilection in the incidence of JDM [5][6][7][8][9][10]. The exact reason is unclear and it is difficult to comment regarding true epidemiological variation of sex ratio in different parts of India or a gender bias bringing more boys for the treatment than the girls [5]. The present study showed a female preponderance similar to western data [13][14][15].…”

“…Higher age at diagnosis has been reported from other parts of India [5-7, 10, 11]. Most of the earlier Indian studies have shown a male predilection in the incidence of JDM [5][6][7][8][9][10]. The exact reason is unclear and it is difficult to comment regarding true epidemiological variation of sex ratio in different parts of India or a gender bias bringing more boys for the treatment than the girls [5].…”

“…The mean time from the onset of symptoms to diagnosis was 5.2 mo (range 1 mo to 18 mo) which is lower than other parts of India [5][6][7][8][9][10][11]. Early diagnosis is documented in western data due to prompt recognition and referral to the specialized center [13,16].…”

“…Studies on IIM from India are scarce in literature and only a few studies have been published from various parts of India that include only JDM [5][6][7][8][9][10][11]. A scarcity of reported data on IIM from the eastern part of India prompted the authors to carry out this retrospective study in a tertiary referral center.…”

Anasarca as the initial presentation of juvenile polymyositis: an uncommon occurrence

“…It is rare below the age of 18 years [1]. Unlike dermatomyositis, which is common in pediatric age group, skin involvement is lacking in polymyositis [2,3]. We report an 8-year-old boy who presented with anasarca as the initial complaint.…”