Update on Sclerosing Cholangitis in Critically III Patients

BACKGROUND: ‛Sclerosing cholangitis in critically ill patients' (SC-CIP) is a cholestatic liver disease of unknown etiology and represents the most prevalent form of secondary sclerosing cholangitis. METHODS: This overview is based on a systematic review of the literature searching for ‘secondary sclerosing cholangitis’, ‘SC-CIP’, ‘cast syndrome’, and ‘ischemic cholangitis’ in the database PubMed. RESULTS: SC-CIP can develop in patients with sepsis and acute respiratory distress syndrome during a long-term intensive care unit (ICU) treatment. It is a rare cholestatic liver disease with a rapid progression to liver cirrhosis and hepatic failure. SC-CIP is initiated by an ischemic injury to the biliary tree with subsequent stenoses of biliary ducts, biliary casts, and infections, often with multi-resistant bacteria. Mechanical ventilation with high positive end-expiratory pressure, prone positioning, and a higher volume of intraperitoneal fat have been proposed as risk factors for developing SC-CIP. Patients with SC-CIP have a poor prognosis, with liver transplantation (LT) being the only curative treatment option. CONCLUSION: In patients with sepsis, long-term ICU therapy and ongoing cholestasis SC-CIP must be excluded by endoscopic retrograde cholangiopancreatography. Due to the poor prognosis, the option of LT should be evaluated in all patients with SC-CIP.