Poor outcome in progressive sclerosing cholangitis after septic shock

Progressive sclerosing cholangitis after septic shock is an increasingly diagnosed disease entity. We evaluated the outcome after long-term follow-up of 29 patients treated in our institution between 1995 and 2007.

Patients with cholestatic liver disease without evidence of pre-existing hepatobiliary disease and who previously required long-term treatment in an intensive care unit for septic shock due to following reasons were included in the study: severe trauma (n = 10; five with burn injury and five following accident), cardiac operation (n = 9), bacterial infection (n = 5), sigmoidectomy (n = 2), operation of aortic aneurysm (n = 3).

In all patients, endoscopic retrograde cholangiopancreatography showed multiple stenoses, pre-stenotic dilatations, and in part rarefication of intrahepatic small bile ducts. The bile ducts were partially filled by black-pigmented or necrotic material. In 18 of 29 patients, liver biopsies were performed and showed fibrosing cholangitis. The endoscopic therapy comprised removal of occluding material, dilation of stenoses, and intermittent stenting if necessary. All endoscopic procedures were done under antibiotic prophylaxis. During follow-up, 19 of the 29 patients died. Three patients received orthotopic liver transplantation. Four patients have been registered for transplantation, and the remaining three patients show signs of severe cholestasis. The actuarial estimate (Kaplan-Meier) indicated a survival free of liver transplantation of 55 % after 1 year, and only 14 % after 6 years. The median survival was 1.1 years.

Progressive sclerosing cholangitis after septic shock is a recently described disease characterized by extremely short survival free of liver transplantation. This disease should be considered in patients who develop cholestasis following treatment of septic shock in an intensive care unit.