IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis. (25th August 2015)

• Record Type: Journal Article
• Authors: Kahn, Allon; Yadav, Anitha D.; Harrison, M. Edwyn
• Other Names: Sugimura Haruhiko Academic Editor.
• Is Part Of: Case reports in gastrointestinal medicine. Volume 2015(2015)
• Publication Date: 2015-08-25
• Subjects: Gastroenterology -- Case studies -- Periodicals; Digestive organs -- Diseases -- Periodicals; Gastroenterology; Gastrointestinal Diseases; Digestive organs -- Diseases; Gastroenterology; Electronic journals; Periodicals; Case studies; Periodicals; 616.33
• DOI: 10.1155/2015/591360
• Languages: English
• ISSNs: 2090-6528

Abstract

Abstract : IgG4-related disease is a relatively novel clinical entity whose gastrointestinal manifestations include type 1 autoimmune pancreatitis (AIP) and IgG4-associated sclerosing cholangitis. The presence of elevated serum IgG4 is suggestive but not essential for the diagnosis of type 1 AIP and is a pervasive feature of the proposed diagnostic criteria. The differential diagnosis of type 1 AIP includes malignant conditions, emphasizing the importance of a deliberate, comprehensive evaluation. Management of patients with a suggestive clinical presentation, but without serum IgG4 elevation, is difficult. Here we present three cases of IgG4-seronegative AIP and sclerosing cholangitis that responded to empiric steroid therapy and discuss approach considerations. These cases demonstrate the value of meticulous application of existing diagnostic algorithms to achieve a clinical diagnosis and avoid surgical intervention.