title: [IgG4-related disease].
abstract: IgG4-related disease is an emerging pathological condition characterized by one or several fibrosing and inflammatory organ involvements. Histological findings are typical and associate storiform fibrosis with polyclonal lymphocytic and plasma-cell infiltrate, with predominant IgG4-expressing plasma cells. Sclerosing lymphoplasmocytic pancreatitis, or type 1 auto-immune pancreatitis, and other organ involvements have been reported: sclerosing cholangitis, sialadenitis, dacryoadenitis, retroperitoneal fibrosis, aortitis, interstitial nephritis, polyadenopathy and inflammatory pseudo-tumors. Serum IgG4 level is elevated in most of patients, but the histological documentation remain necessary for the diagnosis. Dramatic response to steroids is usual but relapses are frequent.
authors.affiliation: Service de médecine interne, hôpital de la Conception, Assistance publique-Hôpitaux de Marseille, 13385 Marseille Cedex 5, France. mikael.ebbo@ap-hm.fr
authors.forename: Mikaël
authors.initials: M
authors.lastname: Ebbo
authors.forename: Aurélie
authors.initials: A
authors.lastname: Grados
authors.forename: Nicolas
authors.initials: N
authors.lastname: Schleinitz
mesh_terms: D001327:Autoimmune Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology* / Q000628:therapy; D002761:Cholangitis / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology / Q000628:therapy; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology / Q000502:physiology*; D008954:Models, Biological; D009395:Nephritis, Interstitial / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology / Q000628:therapy; D010195:Pancreatitis / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000628:therapy; D012185:Retroperitoneal Fibrosis / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000628:therapy; D012598:Sclerosis / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology / Q000628:therapy; D012793:Sialadenitis / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000628:therapy
chemical_list: D007074:Immunoglobulin G