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KMID : 0391520160240040232
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Journal of the Korean Child Neurology Society
2016 Volume.24 No. 4 p.232 ~ p.239
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Clinical Characteristics and Treatment Response of Juvenile Dermatomyositis
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Woo Hye-Won
Cho Jae-So
Kim Soo-Yeon
Choi Sun-Ah
Kim Hun-Min
Lim Byung-Chan
Hwang Hee
Choi Ji-Eun
Kim Ki-Joong
Chae Jong-Hee
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Abstract
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Purpose: Juvenile dermatomyositis (JDM) is the most common form of the inflammatory myopathies in children. This study was aimed to describe clinical characteristics and prognostic factors of JDM.
Methods: We retrospectively reviewed 25 patients who were diagnosed as JDM based on Bohan and Peter criteria at Seoul National University Children’s Hospital from January 2003 to December 2015.
Results: Among 25 patients, 13 (52%) patients were male. The mean age at diagnosis was 7.0 (1.6-13.8) years and the duration from expression of initial symptoms to diagnosis was 4.1(0.0-13.1) months. The follow up duration was 4.8 (0.6-10.8) years and the treatment duration was 3.2 (0.6-9.0) years. Twelve (48%) patients achieved remission, and the relapse rate was 0.3 (0.0-0.8) times/year. In 13 patient of steroid-response group, 10 (77%) patients achieved remission and relapse rate of 0.2 (0.0-0.8) times/year. However, 2 (17%) of 12 patients (P=0.005) obtained remission in steroid-resistance group with relapse rate of 0.4 (0.0-0.7) times/year. In 6 of early-onset group, 1 (17%) patient achieved remission, but 11 (58%) of 19 patients in late-onset group achieved remission. Also, subcutaneous calcinosis was more combined in steroid-resistance group (P=0.002).
Conclusion: Comparing to adult dermatomyositis, treatment response and prognosis of JDM might be more favorable. On the basis of our observations, steroid resistance at initial treatment, high relapse rate, earlier age of onset less than 3 years old, and presence of subcutaneous calcinosis was poor prognostic factors. For confirmative conclusion, further study in large numbers of JDM cohort will be needed.
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KEYWORD
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Juvenile Dermatomyositis, Corticosteroid, Prognosis, Relapse, Age of Onset, Lipodystrophy
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