title: Accumulation of mature B cells in the inflamed muscle tissue of a patient with anti-155/140 antibody-positive juvenile dermatomyositis.
abstract: We report the first case of a Japanese patient with anti-155/140 antibody-positive juvenile dermatomyositis (JDM). Her clinical features included severe cutaneous involvement. Serum B cell-activating factor levels were significantly increased. Mature class-switched memory B cells accumulated in inflamed muscle tissue but decreased in peripheral blood. These findings indicate that loss of B cell tolerance and accumulation of mature B cells in inflamed muscle tissue play an important role in the pathogenesis of JDM.
authors.affiliation: Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, 13-1 Takaramachi, Kanazawa, 920-8641, Japan.
authors.forename: Tadafumi
authors.initials: T
authors.lastname: Yokoyama
authors.forename: Masaki
authors.initials: M
authors.lastname: Shimizu
authors.forename: Sayaka
authors.initials: S
authors.lastname: Ishikawa
authors.forename: Kazuyuki
authors.initials: K
authors.lastname: Ueno
authors.forename: Yasuhito
authors.initials: Y
authors.lastname: Hamaguchi
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Takehara
authors.forename: Akihiro
authors.initials: A
authors.lastname: Yachie
mesh_terms: D000293:Adolescent; D000974:Antibodies, Antinuclear / Q000097:blood*; D001324:Autoantigens / Q000276:immunology; D053264:B-Cell Activating Factor / Q000097:blood; D053301:B-Cell Maturation Antigen / Q000097:blood; D001402:B-Lymphocytes / Q000276:immunology / Q000473:pathology*; D003882:Dermatomyositis / Q000276:immunology / Q000473:pathology*; D005260:Female; D006801:Humans; D007108:Immune Tolerance; D018482:Muscle, Skeletal / Q000276:immunology / Q000473:pathology*; D009687:Nuclear Proteins / Q000276:immunology*; D012074:Remission Induction; D016896:Treatment Outcome
chemical_list: D000974:Antibodies, Antinuclear; D001324:Autoantigens; D053264:B-Cell Activating Factor; D053301:B-Cell Maturation Antigen; D009687:Nuclear Proteins