title: The Geoepidemiology and Clinical Aspects of IgG4-Related Disease.
abstract: Immunoglobulin G4-related disease (IgG4-RD) is a recently described systemic inflammatory disease characterized by increased serum IgG4 concentrations, lymphoplasmacytic infiltrations, storiform fibrosis, and obliterative phlebitis. However, although IgG4-RD has become increasingly recognized, the number of patients with IgG4-RD remains unclear. Data from several studies indicate that patients who have a T-helper type 2 (Th2-) dominant immune response, which leads to the hyperproduction of Th2 cytokines, then progress to IgG4-RD. Glucocorticoids are the most common treatment for IgG4-RD and generally, patients have a good response-a characteristic of IgG4-RD. However, relapses during the tapering of glucocorticoid therapy are common. Second-line therapy after glucocorticoids includes immunosuppressant agents. Although the long-term outcome still remains unclear, there is increased interest in the relationships between IgG-RD and malignancies. In this review, the authors provide a detailed overview of the geoepidemiology, pathogenesis, diagnostic features, treatment, and prognosis of IgG4-RD.
authors.affiliation: Department of Gastroenterology and Hepatology, Kansai Medical University, Hirakata, Osaka, Japan.
authors.forename: Kazushige
authors.initials: K
authors.lastname: Uchida
authors.affiliation: Department of Gastroenterology and Hepatology, Kansai Medical University, Hirakata, Osaka, Japan.
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Tanaka
authors.affiliation: Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, Davis, California.
authors.forename: M Eric
authors.initials: ME
authors.lastname: Gershwin
authors.affiliation: Department of Gastroenterology and Hepatology, Kansai Medical University, Hirakata, Osaka, Japan.
authors.forename: Kazuichi
authors.initials: K
authors.lastname: Okazaki
mesh_terms: D001327:Autoimmune Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology*; D015415:Biomarkers / Q000097:blood; D002908:Chronic Disease; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood* / Q000276:immunology; D007249:Inflammation / Q000453:epidemiology / Q000276:immunology; D007564:Japan / Q000453:epidemiology; D008297:Male; D015995:Prevalence; D012008:Recurrence
chemical_list: D015415:Biomarkers; D005938:Glucocorticoids; D007074:Immunoglobulin G