title: Juvenile dermatomyositis: immunopathogenesis, role of myositis-specific autoantibodies, and review of rituximab use.
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.
authors.affiliation: Division of Pediatric Dermatology, Department of Dermatology, Medical College of Wisconsin Milwaukee, Wisconsin 53226, USA. ychiu@mcw.edu
authors.forename: Yvonne E
authors.initials: YE
authors.lastname: Chiu
authors.forename: Dominic O
authors.initials: DO
authors.lastname: Co
mesh_terms: D000604:Amino Acyl-tRNA Synthetases / Q000276:immunology; D000818:Animals; D058846:Antibodies, Monoclonal, Murine-Derived / Q000009:adverse effects / Q000627:therapeutic use*; D001323:Autoantibodies / Q000097:blood / Q000235:genetics / Q000276:immunology*; D001402:B-Lymphocytes / Q000276:immunology; D002648:Child; D046528:Chimerism; D002986:Clinical Trials as Topic; D003713:Dendritic Cells / Q000276:immunology; D003882:Dermatomyositis / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D007155:Immunologic Factors / Q000009:adverse effects / Q000627:therapeutic use*; D008297:Male; D051379:Mice; D000069283:Rituximab; D018271:Signal Recognition Particle / Q000276:immunology; D013601:T-Lymphocytes / Q000276:immunology; D014157:Transcription Factors / Q000276:immunology
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D007155:Immunologic Factors; C045760:Mi-2 antibodies; D018271:Signal Recognition Particle; C511956:TRIM33 protein, human; D014157:Transcription Factors; D000069283:Rituximab; D000604:Amino Acyl-tRNA Synthetases