IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis

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.

Immunoglobulin G4-Seronegative Autoimmune Cholangiopathy With Pancreatic and Hepatic Involvement Mimicking as Primary Sclerosing Cholangitis

“…The difficulty in identifying a correct diagnosis is exacerbated by the low incidence rate of PSC 2 (0.5-1.3 cases per 100,000 person-years) and significantly rarer incidence of IgG4-seronegative AIC. 3,4 Ten years after initial presentation, a correct diagnosis was made for this patient, allowing initiation of therapy that normalized liver tests and improved biliary strictures. He has improved his quality of life substantially and has for now delayed proceeding to liver transplantation.…”

“…This case highlights the challenges of diagnosing IgG4seronegative AIC and the difficulty in differentiating PSC from other causes of secondary SC and malignancy. 3 Traditionally, the gold standard for diagnosis of PSC is ERCP showing segmental fibrosis within both intrahepatic and extrahepatic bile ducts separated by normal areas of saccular dilatation, commonly referred to as "beads on a string." 1 Despite both magnetic resonance imaging and ERCP showing classic PSC findings which were confirmed by liver biopsy with negative IgG4 staining and normal serum IgG4 levels, it was not until a surveillance MRCP demonstrated inflammation and fibrosis of the pancreatic tail, that the differential of IgG4-seronegative AIC with pancreatic involvement was entertained.…”

“…This case highlights the challenges of diagnosing IgG4-seronegative AIC and the difficulty in differentiating PSC from other causes of secondary SC and malignancy. 3 Traditionally, the gold standard for diagnosis of PSC is ERCP showing segmental fibrosis within both intrahepatic and extrahepatic bile ducts separated by normal areas of saccular dilatation, commonly referred to as “beads on a string.” 1…”

“…The difficulty in identifying a correct diagnosis is exacerbated by the low incidence rate of PSC 2 (0.5–1.3 cases per 100,000 person-years) and significantly rarer incidence of IgG4-seronegative AIC. 3,4…”

IgG4 Sclerosing Cholangitis and Post Infantile Giant Cell Hepatitis: A Case Report of an Extraordinary Co-presentation

“…Interplay between IgG4 positive tissue immunostaining, along with both clinical and cholangiographic features of cholangiopathy, is required for proper diagnosis [5]. Histopathological discrimination between IgG4-SC and cholangiocarcinoma is essential; however, this is not usually easy with the difficulty in the endoscopic transpapillary bile duct biopsy [6].…”