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.
© 2011 Wiley Periodicals, Inc.
MeSH terms
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Amino Acyl-tRNA Synthetases / immunology
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Animals
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Antibodies, Monoclonal, Murine-Derived / adverse effects
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Antibodies, Monoclonal, Murine-Derived / therapeutic use*
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Autoantibodies / blood
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Autoantibodies / genetics
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Autoantibodies / immunology*
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B-Lymphocytes / immunology
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Child
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Chimerism
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Clinical Trials as Topic
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Dendritic Cells / immunology
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Dermatomyositis / drug therapy*
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Dermatomyositis / genetics
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Dermatomyositis / immunology
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Female
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Genetic Predisposition to Disease
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Humans
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Immunologic Factors / adverse effects
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Immunologic Factors / therapeutic use*
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Male
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Mice
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Rituximab
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Signal Recognition Particle / immunology
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T-Lymphocytes / immunology
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Transcription Factors / immunology
Substances
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Antibodies, Monoclonal, Murine-Derived
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Autoantibodies
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Immunologic Factors
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Mi-2 antibodies
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Signal Recognition Particle
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TRIM33 protein, human
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Transcription Factors
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Rituximab
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Amino Acyl-tRNA Synthetases
Supplementary concepts
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Amyopathic dermatomyositis