Juvenile dermatomyositis in north India.

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.