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Twelve years experience of juvenile dermatomyositis in North India
1Department of Paediatrics, Advanced Paediatric Center, Post Graduate Institute of Medical Education and Research, Chandigarh, India, 160012. surjitsinghpgi@rediffmail.com
Insights
Juvenile dermatomyositis (JDMS) outcomes in North India show a distinct clinical profile. Early, aggressive steroid treatment is key to minimizing long-term complications in children with this autoimmune condition.
Area of Science:
- Pediatric Rheumatology
- Autoimmune Diseases
- Clinical Outcomes Research
Background:
- Juvenile dermatomyositis (JDMS) is a rare autoimmune disease affecting children.
- Understanding long-term outcomes and treatment responses in diverse populations is crucial for effective management.
- Previous studies on JDMS outcomes have primarily focused on Western populations.
Purpose of the Study:
- To evaluate the long-term outcomes of children diagnosed with juvenile dermatomyositis (JDMS) at a tertiary care center in North India.
- To analyze the interval between symptom onset and diagnosis, treatment modalities, and functional outcomes.
- To compare the clinical profile of Indian JDMS patients with those reported in Western literature.
Main Methods:
- Retrospective review of medical records for children diagnosed with JDMS over a 13-year period.
- Data collection included symptom onset, diagnosis interval, treatment regimens, and long-term functional status.
- Analysis of complications such as lipodystrophy, calcinosis, dysphagia, and contractures.
Main Results:
- Thirty-three JDMS patients were included, with a mean age at diagnosis of 8.7 years.
- A significant delay in diagnosis was observed (mean 1.18 years).
- Common complications included lipodystrophy (30.3%), calcinosis (27.3%), and cutaneous ulcers (18.2%). Patients receiving adequate initial steroid doses showed sustained response.
Conclusions:
- Early and aggressive immunosuppressive therapy, particularly adequate corticosteroid dosing, is vital for successful JDMS management and minimizing long-term sequelae.
- JDMS patients in North India exhibit a potentially different clinical profile compared to Western cohorts.
- Stepwise, aggressive treatment strategies are highly successful in controlling muscle inflammation and improving long-term functional outcomes.
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.
