A Curious Case of Autoimmune Pancreatitis with IgG4 Related Sclerosing Cholangitis

Abstract

IgG4-related sclerosing cholangitis (IgG4-SC) is an uncommon condition recently recognized as an independent disease entity in hope to better specify future diagnosis modalities and treatment. Its exact pathophysiologic mechanism has yet to be identified, but evidence of submucosal fibrosis and inflammatory cells infiltrating biliary ducts have been noted on biopsies. We present a case of IgG4 related ascending cholangitis in a patient with obstructive jaundice secondary to autoimmune pancreatitis. Patient is a 54 year-old male admitted to the hospital in July 2016 with symptoms of obstructive jaundice. MRI abdomen revealed possible mass on pancreas with a sausage-like appearance of the organ. LFTs were elevated, CEA was 652.9, and IgG4 was 464. Liver biopsy was consistent with IgG4 autoimmune pancreatitis. The patient responded well to steroids and the CEA levels dropped. He followed up in office and steroids were tapered in February 2017 with plans to start Azathioprine. In December 2017 he went to the hospital for obstructive jaundice and RUQ pain. IgG4 was >700. MRI showed relapse of AIP with cystic changes at the level of the pancreatic neck as well as a 1 cm long stricture of the proximal intrapancreatic portion of the CBD and wall thickening of the common hepatic duct and CBD, findings indicative of sclerosing cholangitis or IgG4 related sclerosing disease. The patient was again started on steroids and is currently doing well. IgG4-SC is a novel entity which has been documented to be associated with conditions such as autoimmune pancreatitis, primary sclerosing cholangitis, and cholangiocarcinoma. There is increased prevalence in men, the elderly, and patients with multiorgan involvement. Several factors have been used for diagnosis, such as a serum IgG4 levels, cholangiography, and IgG4-positive plasma cell infiltration on biopsy. Despite the fact that little is known regarding treatment options, IgG4-SC has been documented to respond to steroids. The precise dose of steroids to be utilized has yet to be established. Alternative treatment modalities consist of biliary stent placement, drainage, or surgical intervention. To date, several diagnostic criteria have been proposed as means to help identify IgG4-SC, and other related diseases. It is evident that further comparative diagnostic and treatment studies need to be conducted, as treatment remains inconsistent due to a lack of standardized criteria.