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Juvenile dermatomyositis: immunopathogenesis, role of myositis-specific autoantibodies, and review of rituximab use. Pediatr Dermatol 2011;28(4):357-67

Date

07/29/2011

Pubmed ID

21793879

DOI

10.1111/j.1525-1470.2011.01501.x

Scopus ID

2-s2.0-79960803762 (requires institutional sign-in at Scopus site)   38 Citations

Abstract

Juvenile dermatomyositis (JDM) is an autoimmune disease of the skin and muscle that affects children. The etiology is poorly understood, but genetic susceptibility, environmental triggers, and abnormal immune responses are each thought to play a part. T cells have traditionally been implicated in the immunopathogenesis of JDM, but dendritic cells, B cells, and microchimerism are increasingly associated. Additionally, myositis-specific autoantibodies (MSA) can be present in the sera of affected patients and may correlate with distinct clinical phenotypes. Given the role of humoral immunity and MSA, there has been recent interest in the use of rituximab to treat JDM. Early results are mixed, but it is hoped that a prospective clinical trial will shed light on the issue in the near future.

Author List

Chiu YE, Co DO

Author

Yvonne E. Chiu MD Vice Chair, Professor in the Dermatology department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Amino Acyl-tRNA Synthetases
Animals
Antibodies, Monoclonal, Murine-Derived
Autoantibodies
B-Lymphocytes
Child
Chimerism
Clinical Trials as Topic
Dendritic Cells
Dermatomyositis
Female
Genetic Predisposition to Disease
Humans
Immunologic Factors
Male
Mice
Rituximab
Signal Recognition Particle
T-Lymphocytes
Transcription Factors