S3484 A Case of Ulcerative Proctitis in a Patient With Recent Diagnosis of Autoimmune Pancreatitis

Abstract

INTRODUCTION: The association between autoimmune pancreatitis (AIP) and inflammatory bowel disease (IBD) is extremely rare and poorly understood. Autoimmune pancreatitis presenting in patients with IBD poses a diagnostic dilemma, as it is difficult to differentiate between AIP and primary sclerosing cholangitis. The relationship between IBD and AIP is mostly limited to UC. The following is a rare case of a patient with AIP and UC. CASE DESCRIPTION/METHODS: A 28-year-old man presented to our office for evaluation of abnormal liver function tests. Symptoms included jaundice and an episode of generalized abdominal pain two weeks before, which resolved spontaneously. Remarkable laboratory results included AST 158, ALT 355, ALP 281 and total bilirubin 4.76. MRCP revealed CBD dilation. Subsequent ERCP with cholangioscopy revealed a distal CBD stricture. A directed biopsy was obtained with deployment of a plastic biliary stent for relief of obstruction. Due to his young age and presentation with biliary stricture and a history of abdominal pain, AIP was suspected. IgG4 serology was found to be positive confirming the diagnosis. He was started on steroid therapy and presented later with rectal bleeding prompting colonoscopy evaluation revealing distal inflammation in the rectum with biopsies consistent with ulcerative proctitis. He was treated with oral and rectal mesalamine and a tapering dose of steroids. Two months later his symptoms resolved, and repeat ERCP revealed resolution of the CBD stricture. Follow-up liver enzymes normalized. DISCUSSION: While an association between AIP and concurrent immune/inflammatory diseases, especially UC has been reported, an exact relationship has not been ascertained. Type 2 AIP was recently identified to be associated with IBD. However, Type 2 usually presents in young patients with IgG4 negative serology. This case is unique as our patient is young but presented with IgG4 positive AIP.