title: Immunoglobulin G4-related gastrointestinal diseases, are they immunoglobulin G4-related diseases?
abstract: In immunoglobulin G4 (IgG4)-related disease (RD), organ enlargement or nodular lesions consisting of abundant infiltration of lymphocytes and IgG4-positive plasma cells and fibrosis are seen in various organs. Although infiltration of many IgG4-positive plasma cells is detected in the gastric and colonic mucosa and major duodenal papilla of patients with autoimmune pancreatitis, it cannot be diagnosed as a gastrointestinal lesion involved in IgG4-RD, because none of the following is observed in these lesions: a mass-like formation; dense fibrosis; or obliterative phlebitis. Based on our review of the literature, there appear to be two types of IgG4-related gastrointestinal disease. One is a gastrointestinal lesion showing marked thickening of the wall of the esophagus and stomach, consisting of dense fibrosis with abundant infiltration of IgG4-positive plasma cells, which usually show submucosal spreading. The other is an IgG4-related pseudotumor occurring in gastrointestinal regions such as the stomach, colon, and major duodenal papilla, showing polypoid or mass-like lesions. Most solitary IgG4-related gastrointestinal lesions that are not associated with other IgG4-RD appear to be difficult to diagnose. It is of utmost importance to rule out malignancy. However, these lesions may respond to steroid therapy. To avoid unnecessary resection, IgG4-related gastrointestinal diseases should be considered in the differential diagnosis.
authors.affiliation: Satomi Koizumi, Terumi Kamisawa, Sawako Kuruma, Taku Tabata, Kazuro Chiba, Susumu Iwasaki, Yuka Endo, Go Kuwata, Koichi Koizumi, Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo 113-8677, Japan.
authors.forename: Satomi
authors.initials: S
authors.lastname: Koizumi
authors.forename: Terumi
authors.initials: T
authors.lastname: Kamisawa
authors.forename: Sawako
authors.initials: S
authors.lastname: Kuruma
authors.forename: Taku
authors.initials: T
authors.lastname: Tabata
authors.forename: Kazuro
authors.initials: K
authors.lastname: Chiba
authors.forename: Susumu
authors.initials: S
authors.lastname: Iwasaki
authors.forename: Yuka
authors.initials: Y
authors.lastname: Endo
authors.forename: Go
authors.initials: G
authors.lastname: Kuwata
authors.forename: Koichi
authors.initials: K
authors.lastname: Koizumi
authors.forename: Tooru
authors.initials: T
authors.lastname: Shimosegawa
authors.forename: Kazuichi
authors.initials: K
authors.lastname: Okazaki
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Chiba
mesh_terms: D000818:Animals; D001327:Autoimmune Diseases / Q000276:immunology* / Q000473:pathology / Q000628:therapy; D015415:Biomarkers / Q000032:analysis; D003937:Diagnosis, Differential; D005355:Fibrosis; D005767:Gastrointestinal Diseases / Q000276:immunology* / Q000473:pathology / Q000628:therapy; D041981:Gastrointestinal Tract / Q000276:immunology* / Q000473:pathology; D006104:Granuloma, Plasma Cell / Q000276:immunology / Q000473:pathology; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis*; D008214:Lymphocytes / Q000276:immunology*; D010950:Plasma Cells / Q000276:immunology*; D011237:Predictive Value of Tests
chemical_list: D015415:Biomarkers; D007074:Immunoglobulin G
keywords: Autoimmune pancreatitis; Colonic polyp; Gastritis; Immunoglobulin G4; Ulcerative colitis