Update on Sclerosing Cholangitis in Critically Ill Patients

Abstract

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.

Keywords: Cast syndrome; Sclerosing cholangitis in critically ill patients (SC-CIP); Secondary sclerosing cholangitis.

Fig. 1

a, b Typical endoscopic retrograde cholangiography (ERC) images of two different patients. Intrahepatic bile ducts show multiple irregular strictures, remnants of biliary casts, and rarefaction of the bile ducts. In fig. 1a, a filling defect in the common bile duct is also shown.

Fig. 2

Liver biopsy samples showing morphological features of SC-CIP (portal and parenchymal changes); a-c portal tract showing marked enlargement due to edema, mild mixed inflammatory infiltrate, and mild fibrosis. Adjacent, a periportal bile duct infarct (a, asterisk; periodic acid-schiff stain, 100×) can be seen. Degenerative changes of the bile duct epithelium which included loss of cellular polarity, cellular dropout, and irregularities of the basal membrane (b, hematoxylin and eosin stain, 200×). Hepatocellular and canalicular cholestasis (c, hematoxylin and eosin stain, 200×). Explanted liver: Gross appearance (d) and histological section (e, f) showing severe damage to the large bile duct with cholangiectasis, intraductal bile sludge as well as biliary casts (asterisks; square in d), and segmental ulceration (arrows) of the bile duct epithelium (e, hematoxylin and eosin stain, 10×). Secondary biliary cirrhosis (f, g): progressive periportal and septal fibrosis with bridges linking adjacent portal tracts (f, EVG, 100×; square in e). Marginal bile duct proliferation and ductular metaplasia of periportal hepatocytes with strong expression of cytokeratin 7 (g, 50×; square in e).