Ten-year experience of juvenile dermatomyositis: a retrospective study.

BACKGROUND AND PURPOSE

Juvenile dermatomyositis (JDMS) is a rare multisystemic disease of unknown etiology that primarily affects muscle and skin. This study aimed to evaluate the initial laboratory data, clinical manifestations, complications, and clinical outcomes of patients with JDMS in Taiwan.

METHODS

We reviewed medical charts of patients younger than 18 years with a diagnosis of JDMS at the pediatric department of National Taiwan University Hospital between 1994 and 2004.

RESULTS

A total of 21 patients were included. The female-to-male ratio was 4.25:1. The mean age at onset of symptoms was 6.4 +/- 3.7 years (range, 2 to 14.2 years). The mean age at diagnosis was 6.9 +/- 3.9 years (range, 2.2 to 15 years). Among the initial cutaneous features, Gottron's rash (62%) and facial rash (including malar rash, 62%) were the most common findings. Gottron's rash was also the most common sign (81%) at any time during the disease course. Among other systemic features at disease onset, myalgia (33%) was the most common symptom, followed by arthralgia (19%) and dysphagia (19%). Interstitial pneumonitis was a complication in 2 patients, one of whom was a mortality case. Gastrointestinal tract vasculitis was highly suspected in one patient complicated with duodenal perforation and retroperitoneal abscess. Calcinosis developed in 6 patients (28.5%), and one of these patients presented with disseminated calcinosis. Five of 21 patients achieved drug-free remission for 28 to 94 months from the time they discontinued therapy to the end of the study. Two patients with diagnosis of amyopathic dermatomyositis were also included in this study.

CONCLUSIONS

The long-term outcomes in this study are thought to be good. Because the understanding of the pathogenesis of JDMS is limited, certain complications are still troublesome clinically.