IgG4 Related Sclerosing Cholangitis. (10th November 2014)

• Record Type: Journal Article
• Authors: Joshi, D.; Webster, G. J. M.
• Other Names: DeMorrow Sharon Academic Editor.
• Is Part Of: Advances in hepatology. Volume 2014(2014)
• Publication Date: 2014-11-10
• Subjects: Liver -- Diseases -- Periodicals; Hepatology -- Periodicals; Hepatology; Liver -- Diseases; Liver Diseases; Gastroenterology; Periodicals; 616.3
• DOI: 10.1155/2014/632320
• Languages: English
• ISSNs: 2356-6744

Abstract

Abstract : IgG4 related disease (IgG4-RD) is a multisystemic disorder which has only recently been recognized. IgG4 related sclerosing cholangitis (IgG4-SC) is the biliary manifestation of the disease, often in association with autoimmune pancreatitis (AIP). In this review, we provide an overview of IgG4-RD, with a focus on the biliary manifestations. In particular, we describe the important differential diagnoses of IgG4-SC that need to be considered, namely, primary sclerosing cholangitis (PSC) and cholangiocarcinoma, and provide a management algorithm. Finally, we highlight future directions and unanswered questions which will provide new insights into this exciting and evolving disease entity.