title: IgG4-related sclerosing disease: autoimmune pancreatitis and extrapancreatic manifestations.
abstract: Autoimmune pancreatitis is the pancreatic manifestation of IgG4-related sclerosing disease, which recently was recognized as a distinct disease entity. Numerous extrapancreatic organs, such as the bile ducts, gallbladder, kidneys, retroperitoneum, thyroid, salivary glands, lung, mediastinum, lymph nodes, and prostate may be involved, either synchronously or metachronously. Most cases of autoimmune pancreatitis are associated with elevated serum IgG4 levels; extensive IgG4-positive plasma cells; and infiltration of lymphocytes into various organs, which leads to fibrosis. There are several established diagnostic criteria systems that are used to diagnose autoimmune pancreatitis and that rely on a combination of imaging findings of the pancreas and other organs, serologic findings, pancreatic histologic findings, and response to corticosteroid therapy. It is important to recognize multiorgan involvement of IgG4-related sclerosing disease and be familiar with its clinical and imaging features because it demonstrates a favorable response to treatment.
authors.affiliation: Department of Medical Imaging and Pathology, University of Toronto, Toronto, Canada.
authors.forename: Paraskevi A
authors.initials: PA
authors.lastname: Vlachou
authors.forename: Korosh
authors.initials: K
authors.lastname: Khalili
authors.forename: Hyun-Jung
authors.initials: HJ
authors.lastname: Jang
authors.forename: Sandra
authors.initials: S
authors.lastname: Fischer
authors.forename: Gideon M
authors.initials: GM
authors.lastname: Hirschfield
authors.forename: Tae Kyoung
authors.initials: TK
authors.lastname: Kim
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D001808:Blood Vessels / Q000473:pathology; D002760:Cholangiopancreatography, Endoscopic Retrograde; D049448:Cholangiopancreatography, Magnetic Resonance; D015209:Cholangitis, Sclerosing / Q000000981:diagnostic imaging / Q000209:etiology; D003952:Diagnostic Imaging / Q000379:methods; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D007074:Immunoglobulin G / Q000032:analysis*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D041781:Jaundice, Obstructive / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009928:Organ Specificity; D010195:Pancreatitis / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D010950:Plasma Cells / Q000276:immunology; D011467:Prostate / Q000000981:diagnostic imaging / Q000473:pathology; D012189:Retrospective Studies; D012469:Salivary Glands / Q000473:pathology; D012598:Sclerosis; D014057:Tomography, X-Ray Computed; D014781:Viscera / Q000000981:diagnostic imaging / Q000473:pathology; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents