title: IgG4-related disease.
abstract: IgG4-related disease is a protean condition that mimics many malignant, infectious, and inflammatory disorders. This multi-organ immune-mediated condition links many disorders previously regarded as isolated, single-organ diseases without any known underlying systemic condition. It was recognised as a unified entity only 10 years ago. Histopathology is the key to diagnosis. The three central pathology features of IgG4-related disease are lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis. The extent of fibrosis is an important determinant of responsiveness to immunosuppressive therapies. IgG4-related disease generally responds to glucocorticoids in its inflammatory stage, but recurrent or refractory cases are common. Important mechanistic insights have been derived from studies of patients treated by B-cell depletion. Greater awareness of this disease is needed to ensure earlier diagnoses, which can prevent severe organ damage, disabling tissue fibrosis, and even death. Identification of specific antigens and T-cell clones that drive the disease will be the first steps to elucidate the pathogenesis of IgG4-related disease.
authors.affiliation: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan.
authors.forename: Terumi
authors.initials: T
authors.lastname: Kamisawa
authors.affiliation: Department of Pathology, Kobe University, Kobe, Japan.
authors.forename: Yoh
authors.initials: Y
authors.lastname: Zen
authors.affiliation: Center for Cancer Research, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: Shiv
authors.initials: S
authors.lastname: Pillai
authors.affiliation: Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA. Electronic address: jhstone@mgh.harvard.edu.
authors.forename: John H
authors.initials: JH
authors.lastname: Stone
mesh_terms: D005260:Female; D005355:Fibrosis / Q000473:pathology; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007154:Immune System Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology*; D007074:Immunoglobulin G / Q000276:immunology*; D008214:Lymphocytes / Q000473:pathology; D008297:Male; D010689:Phlebitis / Q000473:pathology; D010950:Plasma Cells / Q000473:pathology
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G