Progressive sclerosing cholangitis after septic shock: a new variant of vanishing bile duct disorders

Abstract

Background: We present nine patients with progressive sclerosing cholangitis after septic shock.

Patients: All nine patients had previously required long term treatment in an intensive care unit for septic shock: two patients with polytrauma, five with burn injury, and two with extensive surgery. They were admitted to our hospital because of cholangitis. Endoscopic retrograde cholangiography revealed severe intrahepatic stenoses in all patients and liver biopsies showed typical signs of sclerosing cholangitis. No patient had pre-existing liver disease.

Results: Mean follow up time was 35 months. In patients with major bile duct stenoses (3/9), 12 endoscopic dilations were performed in total. In one patient, concrements were extracted and intermittent stenting was necessary. To date, 4/9 patients have rapidly developed liver cirrhosis. During follow up, 5/9 patients died: two after fulminant cholangitis, one after liver failure, one due to liver transplantation associated problems, and one after cerebral ischaemia. One patient has been registered for transplantation and the remaining three patients show no acute signs of liver failure.

Conclusions: Patients with sclerosing cholangitis, following septic shock, represent a new variant of vanishing bile duct disorders. In such patients liver disease rapidly progresses to cirrhosis. Endoscopic treatment may only transiently improve the course of the disease. Orthotopic liver transplantation is indicated in end stage disease.

Figure 1

Liver biopsy of patient No 9. Immunostaining for cytokeratin 7. Ductules express cytokeratin 7. Some hepatocytes express bile duct cytokeratin 7, showing bile duct proliferations arising from ductular metaplasia of periportal hepatocytes.

Figure 2

Liver biopsy of patient No 9. Staining with Masson-Goldner showed periportal fibrosis and a chronic inflammatory infiltrate with some neutrophils.

Figure 3

Patient No 1. (A) Endoscopic retrograde cholangiopancreaticography (ERCP) showing multiple strictures in the intrahepatic biliary system. (B) Clinical course with laboratory data. EPT, endoscopic sphincterotomy, CHE, cholecystectomy, AP, alkaline phosphatase.

Figure 3

Patient No 1. (A) Endoscopic retrograde cholangiopancreaticography (ERCP) showing multiple strictures in the intrahepatic biliary system. (B) Clinical course with laboratory data. EPT, endoscopic sphincterotomy, CHE, cholecystectomy, AP, alkaline phosphatase.

Figure 4

Patient No 5. (A) Endoscopic retrograde cholangiopancreaticography (ERCP) showing multiple intrahepatic strictures in the biliary system. (B) Clinical course with laboratory data. AP, alkaline phosphatase.

Figure 4

Patient No 5. (A) Endoscopic retrograde cholangiopancreaticography (ERCP) showing multiple intrahepatic strictures in the biliary system. (B) Clinical course with laboratory data. AP, alkaline phosphatase.

Figure 5

Patient No 7. (A) Endoscopic retrograde cholangiopancreaticography (ERCP) showing intrahepatic strictures and dominant stenoses in the right biliary system, the common bile duct shows irregularities of diameter. (B) Clinical course with laboratory data. L, lithotripsy, D, dilatation, E, endoprosthesis, OLT, orthotopic liver transplantation; AP, alkaline phosphatase.

Figure 5

Patient No 7. (A) Endoscopic retrograde cholangiopancreaticography (ERCP) showing intrahepatic strictures and dominant stenoses in the right biliary system, the common bile duct shows irregularities of diameter. (B) Clinical course with laboratory data. L, lithotripsy, D, dilatation, E, endoprosthesis, OLT, orthotopic liver transplantation; AP, alkaline phosphatase.