Juvenile dermatomyositis: Clinical profile and disease course in 25 patients

R. Shehata¹, S. Al-Mayouf¹, A. Al-Dalaan², A. Al-Mazaid³, S. Al-Balaa⁴, S. Bahabri¹

Departments of Pediatrics¹ and Medicine², King Faisal Specialist Hospital and Research Centre; Departments of Pediatrics³ and Medicine⁴, King Khalid University Hospital, Riyadh, Saudi Arabia.


ABSTRACT
A retrospective analysis of 25 Arab patients with juvenile dermatomyositis (JDMS) was conducted between 1988 and 1996. The mean age at disease onset was 8.25 years (range 1.5 - 15 yrs) with a male: female ratio of 1.5: 1. The disease duration before diagnosis was 1 - 108 months. Two patients had a family history of JDMS. The clinical features included fever in 14 patients (56%), weight loss in 20 (80%), muscle weakness in all 25 (100%), and muscle pain in 14 (56%). Skin lesions included Gottron¼s papules in 15 patients (60%), heliotrope in 13 (52%), erythematous malar rash in 8 (32%), and pigmentary changes in 12 (48%). Seventeen of the 25 patients had arthralgia (68%) and 16 patients had arthritis (64%). Gastrointestinal symptoms were noted in 19 patients (76 %). Myocarditis with cardiac failure was the initial presentation of 1 patient, while 2 had conduction defect. Twelve patients (48%) had respiratory symptoms. The course of the disease was complicated by calcinosis in 10 patients (40%).
All of the patients were treated with prednisone; 15 were also treated with methotrexate. The duration of follow up ranged from 6 - 108 months (mean 54.5 months). Twenty-three patients improved, including those who had calcinosis at the time of presentation, with a current muscle power of 4/5 in 10 patients (40%) and 5/5 in 13 patients (52%). No deaths were reported in our series and no patients are currently bedridden.

Key words
Juvenile dermatomyositis, calcinosis, prednisone.

Rawia Shehata, MD; Sulaiman Al-Mayouf, MD; Abdulla Al-Dalaan, MD; Abdulla Al-Mazaid, MD; Sulaiman Al-Balaa, MD; Sultan Bahabri, MD.

Please address correspondence and reprint requests to: Dr. Sultan Bahabri, Head, Section of Rheumatology, Department of Pediatrics, King Faisal Specialist Hospital and Research Centre, PO Box 3354, Riyadh 11211, Saudi Arabia.

Received on February 9, 1998; accepted in revised form on July 22, 1998.

Clin Exp Rheumatol 1999; 17: 115-118.
© Copyright Clinical and Experimental Rheumatology 1999.