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Volume 40, Number 1, February 2007

Ten-year experience of juvenile dermatomyositis: a retrospective study


Shih-Kai Chiu, Yao-Hsu Yang, Li-Chieh Wang, Bor-Luen Chiang
Department of Pediatrics, Buddhist Tzu Chi General Hospital, Taipei Branch, and Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan

Received: June 28, 2005    Revised: April 20, 2006    Accepted: May 2, 2006   

 

Corresponding author:

Bor-Luen Chiang, Department of Pediatrics, National Taiwan University Hospital, Fl. 7, No. 7, Chung-Shan S. Road, Taipei 100, Taiwan. E-mail: gicmbor@ha.mc.ntu.edu.tw This e-mail address is being protected from spam bots, you need JavaScript enabled to view it

 



 

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.



 

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.



 

Conclusion:

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.



 

Key words:

Child; Child, preschool; Dermatomyositis; Prognosis; Retrospective studies; Treatment outcome


 



 

J Microbiol Immunol Infect 2007;40:68-73.