A Clinical Study of Juvenile Dermatomyositis

Kim, Seong Ah; Choi, In Kyung; Kim, Tai Seung; Kim, Dong Soo

Abstract

OBJECTIVE
Juvenile dermatomyositis (JDM) is a multisystemic inflammatory disease in which the muscle, skin and gastrointestinal tract are prominently involved. It is characterized early in its course by microvasculopathy of varying severity and later by the development of calcinosis. The purpose of this study is to evaluate the clinical features, laboratory data, and outcome of patients with JDM.
METHODS
We identified 21 patients (8 males and 13 females: average age 7.1+/-4.9 years) diagnosed as JDM at Yonsei Medical Center between March 1986 and August 2001 by retrospective chart review. The diagnosis of JDM was based on the published criteria proposed by Bohan and Peter in 1975.
RESULTS
The average time between the initial symptoms and the diagnosis of JDM was 4.0+/-7.5 months (range, 1~36 months) with the peak of onset in the summer months (June to august, 48%). The most common presenting feature was proximal muscle weakness followed by skin rash. Other symptoms were respiratory symptoms (33%) and calcinosis (10%). Laboratoy abnormalities included elevation of CPK (81%), AST (62%), LDH (43%) and antinuclear antibody (47%). Electromyograms were abnormal in 13 of 16 performed, showing characteristic myopathic abnormalities. Muscle biopsies revealed typical changes in 11 of 17 performed. All patients were treated with prednisolone (1~2 mg/kg/day) as part of their initial therapy, and 6 cases received intravenous high dose methylprednisolone (30 mg/kg/dose for 3 days). Methotrexate (5 cases), cyclosporine (5 cases), hydroxychloroquine (3 cases), and NSAIDs (2 cases) were given as a second drug, if required. Eleven cases had a monocyclic, 5 cases a chronic polycyclic, and 5 cases a chronic continuous course. One fulminant type case died of sepsis secondary to cellulitis on both axilla.
CONCLUSION
The results of our review agree with other studies reported, but our study showed less occurrence of respiratory symptoms and calcinosis than other reports, and showed the peak incidence in the summer months. Given the fact that the number of our sample was small, future multicenter studies will be necessary.