Dermatomyositis and type 1 interferons.
Dermatomyositis is a poorly understood multisystem disease predominantly affecting skin and muscle. This review focuses on the potential role of a group of related cytokines, the type 1 interferons, in the pathogenesis of dermatomyositis. Type 1 interferon-inducible transcripts and proteins are uniquely elevated in dermatomyositis muscle compared with all other muscle diseases studied to date. The endothelial cell tubuloreticular inclusions present in affected dermatomyositis muscle are biomarkers of type 1 interferon exposure. The cell-poor lichenoid reaction in skin with predominant involvement of the basal epidermal cell layer and its topologic equivalent in muscle, perifascicular atrophy, may be lesions that develop directly in response to type 1 interferon signaling.
Greenberg
Steven A
SA
Department of Neurology, Division of Neuromuscular Disease, Brigham and Women's Hospital, 75 Francis Street, Boston, MA 02115, USA. sagreenberg@partners.org
D015415:Biomarkers; D003882:Dermatomyositis / Q000378:metabolism* / Q000473:pathology; D004730:Endothelium, Vascular / Q000378:metabolism / Q000473:pathology; D006801:Humans; D002479:Inclusion Bodies / Q000378:metabolism / Q000473:pathology; D007370:Interferon Type I / Q000235:genetics / Q000378:metabolism*; D018482:Muscle, Skeletal / Q000378:metabolism* / Q000473:pathology; D015398:Signal Transduction; D012867:Skin / Q000378:metabolism* / Q000473:pathology
D015415:Biomarkers; D007370:Interferon Type I