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Immunopathogenesis of juvenile dermatomyositis
Author(s) -
Khanna Sahil,
Reed Ann M.
Publication year - 2010
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.21669
Subject(s) - juvenile dermatomyositis , dermatomyositis , immunology , medicine , innate immune system , major histocompatibility complex , immune system , immune dysregulation , pathogenesis , autoantibody , pathology , antibody
There is increasing evidence for involvement of the mechanisms of the innate immune system in the pathogenesis of idiopathic inflammatory myopathies (IIMs), especially in the adult and juvenile forms of dermatomyositis. Juvenile dermatomyositis (JDM) is the most common form of childhood IIM, and this review focuses on recent advances in understanding the actions of the innate immune system in this condition. Over the last few years, great strides have been made in understanding immune dysregulation in IIM, including JDM. Novel autoantibodies have been identified, and new genetic contributions have been described. Among the most striking findings is type I interferon activity in JDM tissue and peripheral blood. This is in conjunction with the description of dysregulation of the major histocompatibility complex (MHC) class I gene and identification of plasmacytoid dendritic infiltrates as the possible cellular source of type I interferons. These findings also point toward the potential prognostic value of muscle biopsies and have helped expand our understanding of the etiopathogenesis of IIM. Muscle Nerve 41: 581–592, 2010