title: MxA gene expression in juvenile dermatomyositis peripheral blood mononuclear cells: association with muscle involvement.
abstract: Juvenile dermatomyositis (JDM), a systemic vasculopathy, is characterized by inflammation of skin and muscle. Muscle biopsies from untreated JDM patients show upregulation of type I interferon (IFN)-inducible genes, including myxovirus resistance protein A (MxA). The present study examines whether MxA mRNA expression in peripheral blood mononuclear cells (PBMC) from JDM patients: (1) is elevated compared to healthy controls, (2) reflects disease activity, and (3) changes with the onset of clinically effective treatment. MxA mRNA expression in JDM PBMC obtained at the initial clinic visit was elevated compared to controls and was positively correlated with Disease Activity Score (DAS) for muscle, but not with DAS for skin, suggesting that damage to skin and muscle in JDM may each have a discrete pathophysiology. During the course of clinically effective treatment, decrease in muscle symptoms was associated with a decrease in PBMC MxA mRNA expression.
authors.affiliation: Molecular and Cellular Pathobiology Program, Children's Memorial Research Center, Box 212, Chicago, IL 60614, USA. k-oconnor3@northwestern.edu
authors.forename: Kevin A
authors.initials: KA
authors.lastname: O'Connor
authors.forename: Kathy A
authors.initials: KA
authors.lastname: Abbott
authors.forename: Brad
authors.initials: B
authors.lastname: Sabin
authors.forename: Maxine
authors.initials: M
authors.lastname: Kuroda
authors.forename: Lauren M
authors.initials: LM
authors.lastname: Pachman
mesh_terms: D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001219:Aspartate Aminotransferases / Q000097:blood; D002648:Child; D003402:Creatine Kinase / Q000097:blood; D003882:Dermatomyositis / Q000097:blood / Q000188:drug therapy / Q000235:genetics / Q000276:immunology*; D005260:Female; D005634:Fructose-Bisphosphate Aldolase / Q000097:blood; D019204:GTP-Binding Proteins / Q000096:biosynthesis* / Q000097:blood / Q000235:genetics / Q000276:immunology; D006801:Humans; D007770:L-Lactate Dehydrogenase / Q000097:blood; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008775:Methylprednisolone / Q000627:therapeutic use; D018482:Muscle, Skeletal / Q000276:immunology* / Q000378:metabolism / Q000473:pathology; D064547:Myxovirus Resistance Proteins; D012333:RNA, Messenger / Q000096:biosynthesis / Q000097:blood / Q000235:genetics; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012867:Skin / Q000276:immunology / Q000378:metabolism / Q000473:pathology
chemical_list: D000893:Anti-Inflammatory Agents; C579071:MX1 protein, human; D064547:Myxovirus Resistance Proteins; D012333:RNA, Messenger; D007770:L-Lactate Dehydrogenase; D001219:Aspartate Aminotransferases; D003402:Creatine Kinase; D019204:GTP-Binding Proteins; D005634:Fructose-Bisphosphate Aldolase; D008775:Methylprednisolone; D008727:Methotrexate