S2531 A Rare Cause of Cholestasis: Secondary Sclerosing Cholangitis in a Critically Ill Patient

Abstract

INTRODUCTION: Secondary sclerosing cholangitis in critically ill patient (SSC-CIP) is a newly diagnosed entity of severe biliary disease which can rapidly progress to liver cirrhosis. The mechanism leading to this form of cholangitis with stricture formation and complete obliteration of bile ducts is unknown (1). CASE DESCRIPTION/METHODS: 47 year old woman with history of roux en y gastric bypass was admitted for status epilepticus, pneumonia and shock requiring vasopressors and ventilator support for over a month in ICU. Patient’s LFTs at discharge were normal. Patient was readmitted few weeks later with a seizure and found to have abnormal LFTs (ALP 2500, GGT 4000, direct bilirubin 3.2, ALT 80, and AST 75, lipase 80) which persisted over the course of next ten months. The negative work up included HAV, HBV, HCV, HIV, ANA, AMA, ASMA, Immunoglobulins, Anti LKM, ANCA, Anti SP 100, Anti GP 210, ceruloplasmin, alpha 1 anti-trypsin, TTG IgG, TSH, AFP, CA19-9 and leukemia/lymphoma markers. Her ultrasound and CT scan was unremarkable. Patient was unable to get a MRCP due to a vagal nerve stimulator and ERCP was not done due to difficult anatomy. She underwent a IR guided cholangiogram; subtle stricturing and mild dilation of left intrahepatic ducts seen, followed by a liver biopsy. Biopsy showed large bile duct obstruction with secondary sclerosing cholangitis due to ischemic cholangiopathy with advanced biliary fibrosis. Patient was started on ursodiol with improvement of LFTs however she got readmitted few days later with hemorrhagic shock due to delayed bleed from the liver biopsy site with sepsis and multiorgan failure resulting in death. DISCUSSION: SSC-CIP can be caused by events such as prolonged hypotension and respiratory insufficiency that lead to severe irreversible injury of ducts (2). The trigger event of ischemic cholangoipathy in our patient was likely her initial critical illness (hypotension and respiratory failure) resulting in cholestasis. This variant SSC-CIP is a relatively new disease that is an under recognized cause of cholestasis and should be suspected as a differential in patients with new cholestasis after a critical illness.