S1394 A Rare Form of Secondary Sclerosing Cholangitis Seen in Critically Ill Patients

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INTRODUCTION: Secondary sclerosing cholangitis (SSC) is caused by biliary obstruction and damage to the biliary tree often with progression to cirrhosis. There is a rare entity of SSC following resolution of critical illness without preexisting biliary obstruction called “secondary sclerosing cholangitis in critically ill patients” (SSC-CIP). We present a case of SSC-CIP in a patient with respiratory failure from influenza A. CASE DESCRIPTION/METHODS: A 44-year-old man with no preexisting hepatobiliary disease developed respiratory failure requiring intubation secondary to Influenza A. He required pronation as well as veno-venous extracorporeal membrane oxygenation (VV-ECMO). Two months after his respiratory failure resolved he was discharged. Shortly thereafter he returned with sepsis manifest as fever, leukocytosis and an elevated alkaline phosphatase to 1323 IU/L. MRCP demonstrated multifocal hepatic abscesses and irregular appearing right intrahepatic bile ducts with strong enhancement, suspicious for cholangitis (Figure 1). ERCP revealed irregularity and saccular dilation of the right intrahepatic ducts with viscous bile, brown stones, and debris. The extrahepatic common bile duct appeared normal. The endoscopic findings in this clinical context were consistent with SSC-CIP. DISCUSSION: While secondary sclerosing cholangitis most commonly presents with biliary obstruction, SSC-CIP has a unique presentation with hepatic abscesses in the absence of biliary obstruction following resolution of critical illness. Although rare, the majority of cases in the literature develop in patients recently recovered from ARDS secondary to influenza A. Although the pathophysiology underlying SSC-CIP has not been definitively elucidated, it has been postulated that biliary ischemia of the intrahepatic bile ducts supplied by the hepatosplanchnic plexus is the dominant cause of disease development. Given its novelty, it has been suggested that newer therapies in critical care medicine may contribute to SSC-CIP, specifically both lung protective mechanical ventilation for ARDS which includes high PEEP and low tidal volumes as well as prone positioning. These interventions increase intra-abdominal pressure and can lead to decreased hepatosplanchnic blood flow and subsequent biliary ischemia. With the advent of COVID19 we expect to see more patients affected by this entity, and patient care will be enhanced by physician awareness of SSC-CIP.Figure 1.: MRCP showing multifocal intrahepatic biliary ductal dilatation with diffuse irregularity.