MHC class I and II expression in juvenile dermatomyositis skeletal muscle

Clin Exp Rheumatol. May-Jun 2009;27(3):519-26.


Objective: To assess MHC I and II expressions in muscle fibres of juvenile dermatomyositis (JDM) and compare with the expression in polymyositis (PM), dermatomyositis (DM) and dystrophy.

Patients and methods: Forty-eight JDM patients and 17 controls (8 PM, 5 DM and 4 dystrophy) were studied. The mean age at disease onset was 7.1+/-3.0 years and the mean duration of weakness before biopsy was 9.4+/-12.9 months. Routinehistochemistry and immunohistochemistry (StreptABComplex/HRP) for MHC I and II (Dakopatts) were performed on serial frozen muscle sections in all patients. Mann-Whitney, Kruskal Wallis, chi-square and Fisher's exact statistical methods were used.

Results: MHC I expression was positive in 47 (97.9%) JDM cases. This expression was observed independent of time of disease, corticotherapy previous to muscle biopsy and to the grading of inflammation observed in clinical, laboratorial and histological parameters. The expression of MHC I was similar on JDM, PM and DM, and lower in dystrophy. On the other hand, MHC II expression was positive in just 28.2% of JDM cases and was correlated to histological features as inflammatory infiltrate, increased connective tissue and VAS for global degree of abnormality (p<0.05). MHC II expression was similar in DM/PM and lower in JDM and dystrophy, and it was based on the frequency of positive staining rather than to the degree of the MCH II expression.

Conclusions: MHC I expression in muscle fibres is a premature and late marker of JDM patient independent to corticotherapy, and MHC II expression was lower in JDM than in PM and DM.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Biomarkers / metabolism
  • Biopsy
  • Child
  • Child, Preschool
  • Cross-Sectional Studies
  • Dermatomyositis / diagnosis
  • Dermatomyositis / metabolism*
  • Dermatomyositis / pathology
  • Diagnosis, Differential
  • Gene Expression Regulation
  • Genes, MHC Class I / genetics
  • Genes, MHC Class II / genetics
  • HLA Antigens / metabolism*
  • Humans
  • Muscle, Skeletal / metabolism*
  • Muscle, Skeletal / pathology
  • Muscular Dystrophies / diagnosis
  • Muscular Dystrophies / metabolism
  • Muscular Dystrophies / pathology
  • Polymyositis / diagnosis
  • Polymyositis / metabolism
  • Polymyositis / pathology


  • Biomarkers
  • HLA Antigens