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Published on: March 25, 2016
Outcome in juvenile dermatomyositis
Vaidehi Chowdhary1, Anupam Wakhlu, Amita Agarwal
1Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Rae Barielly Road, Lucknow. rnmisra@sgpgi.ac.in
Insights
Early recognition and aggressive treatment of juvenile dermatomyositis (JDM) are crucial for improving patient outcomes. This study highlights key clinical features, treatment responses, and complications in pediatric JDM cases.
Area of Science:
- Pediatric Rheumatology
- Immunology
Background:
- Juvenile dermatomyositis (JDM) is a rare autoimmune disease affecting children.
- Understanding its clinical spectrum, treatment, and complications is vital for effective management.
Purpose of the Study:
- To analyze the clinical features, outcomes, and complications of juvenile dermatomyositis.
- To evaluate treatment responses and identify factors influencing prognosis in JDM patients.
Main Methods:
- Retrospective analysis of case records from a tertiary care hospital.
- Inclusion of 19 patients diagnosed with juvenile dermatomyositis over an 11-year period.
- Review of clinical manifestations, diagnostic delays, treatment regimens, and patient outcomes.
Main Results:
- Proximal muscle weakness, heliotrope rash, and Gottron's rash were common presenting features.
- Complications included calcinosis, contractures, tuberculosis, and pyogenic infections.
- Significant rates of complete and partial remission were observed with prednisolone and methotrexate treatment.
Conclusions:
- Juvenile dermatomyositis requires prompt diagnosis and aggressive therapeutic strategies.
- Early intervention can lead to improved remission rates and reduced long-term complications.
- Multidisciplinary care is essential for managing the diverse clinical manifestations of JDM.
Abstract:
The clinical features, outcome and complications of juvenile dermatomyositis were studied in a tertiary care hospital by retrospective analysis of case records. Nineteen patients were treated over an 11-year period. Median age at diagnosis was 12 years (2.5-16 years). Median duration of disease prior to diagnosis was 12 months (2-96 months). Proximal muscle weakness was seen in all 19 cases, neck muscle weakness in 14, pharyngeal muscle involvement in 5 and respiratory muscle involvement in 3 cases. Heliotrope rash was seen in 9 and Gottrons rash in 8 patients. Myocarditis and GI bleed were seen in 1 each while interstitial lung disease was seen in 2 patients. All except one patient received prednisolone. Methotrexate was used in 13 and azathioprine in 3 patients. Eight patients are in complete remission (CR), 8 partial remission and 2 patients had no response. Complications were calcinosis in 5, contractures in 2, TB in 4 and pyogenic infections in 4 patients. Juvenile dermatomyositis needs to be recognised early and treated aggressively to improve outcome.
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