Association of Diagnostic Criteria and Autoantibodies with 
Juvenile Dermatomyositis in Newly Diagnosed Children

Shin, Kyung Sue; Kim, Joong Gon

Original Article

Journal of the Korean Pediatric Society 2003;46(9):898-902.
Published online September 15, 2003.

Association of Diagnostic Criteria and Autoantibodies with Juvenile Dermatomyositis in Newly Diagnosed Children

¹Department of Pediatrics, College of Medicine, Cheju National University, Jeju, Korea
²Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea

소아기 피부근염의 진단 기준과 자가항체의 진단적 의의

신경수^¹^, 김중곤^²

^¹제주대학교 의과대학 소아과학교실
^²서울대학교 의과대학 소아과학교실

Correspondence:
Kyung Sue Shin, Email: kyungsue@cheju.ac.kr

Abstract

Purpose
: To determine the clinical association of diagnostic criteria and the prevalence of autoantibodies in newly diagnosed children with juvenile dermatomyositis(JDM).
Methods
: Thirty-two children with JDM were identified at Seoul National University Children's Hospital from March 1985 to March 1999 by retrospective review. The diagnosis of JDM was based on the criteria proposed by Bohan and Peter. We investigated for the presence of several autoantibodies: antinuclear(ANA), double-stranded DNA, anti-Sm, anti-ribonucleoprotein(RNP), anti-SSA/ SSB, anti-Jo1, anti-Scl-70 antibodies and rheumatoid factor(RF).
Results
: Sex ratio and age at diagnosis were similar to data published in other studies. All the newly diagnosed children with JDM had a typical rash(100%) and proximal muscle weakness(100%); 17(53%) had muscle pain or tenderness; 10(31%) calcinosis; eight(25%) dysphagia; eight(25%) arthritis, and seven(22%) fever. Muscle enzymes were elevated in 90% of the patients. Of the 27 patients who had an electromyogram, 20(70%) had diagnostic results. Sixteen(70%) of biopsied patients had appropriated results for JDM. Patients were negative for all autoantibodies except ANA and RF. ANA and RF were detected in 47% and 7% of the patients respectively.
Conclusion
: Although the sensitivity of the criteria proposed by Bohan and Peter is superior, each of these criteria has possible confounding factors. Additional criteria may be needed for early diagnosis of JDM. Based on our findings of autoantibodies in JDM, we do not recommend routine testing for autoantibodies in children with typical JDM.

Key Words: Juvenile dermatomyositis, Diagnostic criteria, Autoantibody