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Juvenile dermatomyositis in north India
S Singh1, L Kumar, K R Shankar
1Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh.
Insights
Juvenile dermatomyositis (JDM) patients typically respond well to early corticosteroid therapy. Methotrexate is an option for children with JDM who show limited improvement with prolonged steroid treatment.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Dermatology
Background:
- Juvenile dermatomyositis (JDM) is a rare autoimmune disease affecting children.
- Understanding its clinical presentation and treatment outcomes is crucial for effective management.
Purpose of the Study:
- To characterize the clinical profile of JDM patients.
- To evaluate the therapeutic response to treatments in JDM.
Main Methods:
- A hospital-based descriptive follow-up study was conducted.
- Data from 12 pediatric patients diagnosed with JDM over five years were analyzed.
Main Results:
- Proximal muscle weakness was a universal finding in JDM patients.
- Skin manifestations were common, while muscle pain and vasculitis were infrequent.
- Early corticosteroid therapy (prednisolone) led to excellent clinical responses in most JDM cases.
- Two patients with refractory disease showed improvement with methotrexate.
Conclusions:
- Corticosteroids are effective for JDM, requiring prolonged treatment duration.
- Methotrexate can be a valuable alternative for JDM patients unresponsive to steroids.
Objective:
To determine the clinical profile and therapeutic response of patients with juvenile dermatomyositis (JDM).
Design:
Hospital based descriptive follow-up study.
Sample:
12 patients attending the Pediatric Rheumatology and Immunology Clinic over last five years.
Results:
The patients were aged between 3 1/2 years to 12 years with a male to female ratio of 2:1. All patients had proximal muscle weakness at presentation. Distal muscle weakness and masseter atrophy was seen in 2 patients and neck flexor weakness and pharyngeal weakness was seen in 1 case. Muscle pain, tenderness or swallowing difficulties were not observed. Classical skin manifestations of JDM were present in all except one patient. Vasculitic lesions were not noted. One patient had diffuse lipoatrophy. Two patients developed calcinosis cutis while on treatment. All patients were put on oral corticosteroids (prednisolone 2 mg/kg/day) initially which were gradually tapered while monitoring clinical response. Early initiation of steroid therapy resulted in an excellent response. Two patients who did not show significant improvement even with prolonged steroid therapy were given oral weekly methotrexate (10 mg/m2/week).
Conclusions:
Most of the children with JDM showed good response to steroid therapy which needs to be continued for a prolonged period. Children who do not respond to this therapy may be given oral weekly methotrexate.