title: IgG4-related disease: historical overview and pathology of hematological disorders.
abstract: IgG4-related diseases comprise a recently recognized systemic syndrome characterized by mass-forming lesions in mainly exocrine tissue that consist of lymphoplasmacytic infiltrates and sclerosis. There are numerous IgG4-positive plasma cells in the affected tissues, and the serum IgG4 level is increased in these patients. The present study describes the history, autoimmune pancreatitis (AIP), IgG4-related lymphadenopathy and lymphomagenesis based upon ocular adnexal IgG4-related disease. Lymphoplasmacytic sclerosing pancreatitis, a prototypal histological type of AIP, is now recognized as a systemic IgG4-related disease. Lymph node lesions can be subdivided into at least five histological subtypes, and systemic IgG4-related lymphadenopathy should be distinguished from multicentric Castleman's disease. Interleukin-6 and CRP levels are abnormally high in multicentric Castleman's disease, but are normal in the majority of systemic IgG4-related lymphadenopathy. Ocular adnexal IgG4-related disease frequently involves bilateral lacrimal glands swelling, and obliterative phlebitis is rare. Moreover, some malignant lymphomas, especially mucosa-associated lymphoid tissue lymphoma, arise from ocular adnexal IgG4-related disease. In addition, IgG4-producing lymphoma also exists.
authors.affiliation: Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Kita-ku, Okayama, Japan.
authors.forename: Yasuharu
authors.initials: Y
authors.lastname: Sato
authors.forename: Kenji
authors.initials: K
authors.lastname: Notohara
authors.forename: Masaru
authors.initials: M
authors.lastname: Kojima
authors.forename: Katsuyoshi
authors.initials: K
authors.lastname: Takata
authors.forename: Yasufumi
authors.initials: Y
authors.lastname: Masaki
authors.forename: Tadashi
authors.initials: T
authors.lastname: Yoshino
mesh_terms: D001323:Autoantibodies / Q000276:immunology; D001327:Autoimmune Diseases / Q000276:immunology / Q000473:pathology*; D006402:Hematologic Diseases / Q000276:immunology / Q000473:pathology*; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D008206:Lymphatic Diseases / Q000276:immunology / Q000473:pathology; D010195:Pancreatitis / Q000276:immunology / Q000473:pathology*; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology*; D012598:Sclerosis / Q000276:immunology / Q000473:pathology; D013577:Syndrome
chemical_list: D001323:Autoantibodies; D007074:Immunoglobulin G