title: The clinical spectrum of IgG4-related disease.
abstract: IgG4-related disease (IgG4-RD) is an emerging immune-mediated disease with the capability of involving essentially any organ. The epidemiology of this disease has not been explored in detail. A majority of patients reported in the literature to date are from Japan, but the condition has been described all across the world and there is no strong evidence to suggest a predilection for Asian populations. The mean age at diagnosis is approximately 60 years and there is a decided male predominance for many clinical features, with an overall male:female ratio of 8:3. A cardinal feature of IgG4-RD is single or multiple organ swelling that often raises concern for malignancy. IgG4-RD should be suspected in patients presenting with unexplained enlargement or swelling of one or more organs. Presenting features vary substantially according to the specialty to which patients present first; in addition, the disease can be diagnosed unexpectedly in pathological specimens or identified incidentally on radiology studies. Involvement of major organs is common and IgG4-RD may lead to organ failure, particularly in the pancreas, liver and biliary tree, kidneys, thyroid gland, lungs, and aorta. The diagnosis of IgG4-RD relies on the coexistence of various clinical, laboratory and histopathological findings, although none is pathognomonic by itself.
authors.affiliation: Josep Font Laboratory of Autoimmune Diseases, CELLEX-Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain; Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Spain.
authors.forename: Pilar
authors.initials: P
authors.lastname: Brito-Zerón
authors.affiliation: Josep Font Laboratory of Autoimmune Diseases, CELLEX-Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain; Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Spain.
authors.forename: Manuel
authors.initials: M
authors.lastname: Ramos-Casals
authors.affiliation: Department of Internal Medicine (ICMiD), Hospital Clínic, Barcelona, Spain.
authors.forename: Xavier
authors.initials: X
authors.lastname: Bosch
authors.affiliation: Harvard Medical School and Department of Medicine, Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Boston, MA 02114, USA. Electronic address: jhstone@partners.org.
authors.forename: John H
authors.initials: JH
authors.lastname: Stone
mesh_terms: D001327:Autoimmune Diseases / Q000276:immunology*; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D009928:Organ Specificity
chemical_list: D007074:Immunoglobulin G
keywords: Aortitis; Autoimmune pancreatitis; IgG4-related disease; Mikulicz disease; Retroperitoneal fibrosis; Sclerosing cholangitis