title: Current Concept of IgG4-Related Disease.
abstract: IgG4-related disease (IgG4-RD) is a fibroinflammatory disease of unknown etiology, which is characterized by a tendency to form tumefactive lesions, increased serum levels of IgG4, and massive infiltration of IgG4-positive plasma cells with storiform fibrosis and/or obliterative phlebitis. Patients with IgG4-RD have frequently multiorgan involvements such as the pancreas, biliary tree, salivary glands, periorbital tissues, kidneys, lungs, lymph nodes, and retroperitoneum. IgG4-RD mainly affects middle-aged to elderly men except for involvement in lachrymal and salivary glands, so-called Mikulicz's disease. The clinical manifestations of IgG4-RD depend on individually involved organs and respond well to steroid, but the prognosis still remains unclear. Some patients develop serious complications such as obstructive jaundice due to hepatic, gallbladder, or pancreatic lesions; hydronephrosis due to retroperitoneal fibrosis; or respiratory symptoms due to pulmonary lesions. Nomenclatures of individual organ manifestation of IgG4-RD have been internationally consented.
authors.affiliation: Department of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan. okazaki@hirakata.kmu.ac.jp.|The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Shinmachi, Hirakata, Osaka, 573-1197, Japan. okazaki@hirakata.kmu.ac.jp.
authors.forename: Kazuichi
authors.initials: K
authors.lastname: Okazaki
authors.affiliation: Northern County Center for RA and Autoimmune Disease, Hayashi Hospital, Fukui, Japan.
authors.forename: Hisanori
authors.initials: H
authors.lastname: Umehara
mesh_terms: D000818:Animals; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology*; D007249:Inflammation / Q000097:blood* / Q000276:immunology
chemical_list: D007074:Immunoglobulin G
keywords: Autoimmune pancreatitis; Disease concept; IgG4-related disease