Objectives: Sclerosing cholangitis in critically ill patients (SC-CIP) is a newly described entity of severe biliary disease with progression to liver cirrhosis. The mechanisms leading to this form of cholangiopathy with stricture formation and complete obliteration of bile ducts are unknown.
Patients and methods: In the last 2 yr, sclerosing cholangitis was diagnosed in 26 patients during or after their stay on the intensive care unit by ERCP and/or liver histology. Complete patient records were available for 17 patients. Histological evaluations of liver biopsies and of four explanted livers, parameters of cardiovascular and respiratory conditions, treatment modalities, and accompanying infections were analyzed to find further hints for the pathomechanisms leading to SC-CIP.
Results: With the beginning of cholestasis, the earliest endoscopic findings were intrahepatic biliary casts with impairment of the biliary flow and subsequent biliary infection, in most cases with Enterococcus faecium. Liver biopsy confirmed cholangitis and histology of explanted livers revealed ulcerated biliary epithelium with hemorrhagic exudates in the bile ducts. In the further course, progressive sclerosis with formation of multiple strictures of the bile ducts was observed. All patients suffered severe respiratory insufficiency with the need for mechanical ventilation (40.7+/-32.9 days). The PaO2/FiO2 ratio until beginning of cholestasis was 150.5+/-43.1. Half of the patients (9/17) were treated with high-frequency oscillatory ventilation and 12/17 patients by intermittent prone positioning. All patients required catecholamines for hemodynamic stabilization.
Conclusions: SC-CIP is a severe and in most cases rapidly progressive complication of intensive care patients. Ischemic injury of the biliary tree with the formation of biliary casts and subsequent ongoing biliary infection due to multiresistant bacteria seem to be major pathogenic mechanisms in the development of this new entity of sclerosing cholangitis.