The challenges in primary sclerosing cholangitis – Aetiopathogenesis, autoimmunity, management and malignancy

Typical examples of PSC cholangiograms. (A) Type I intrahepatic alterations, (B) Type II intraheptic alterations, (C) Type III intraheptic alterations.

Secondary biliary sclerosis can mimick cholangiographic features of PSC. (A) The cholangiogram of a patient with ischemic-like cholangiopathy and biliary cast formation after prolonged anamnestic polytrauma with sepsis and mechanical ventilation. (B) A biliary cast that had been removed from the hepatic duct in this patient.

ERC with the corresponding MRC of two patients with PSC. Patient A presents with multifocal strictures of the intrahepatic bile ducts and with a high-grade stenosis at the cystic duct junction. Patient B features a long-segment filiform stenosis of the common bile duct; the intrahepatic ducts seem to be profoundly narrowed in ERC while MRC accentuates the dilated bile ducts in intervening segments.

Cholangiogram of a PSC patient. The arrow indicates a polypoid mass in the hilar region. Forceps biopsy revealed CC.

Cholangiogram (A) and cholangioscopic appearance (B) of a benign stricture in a patient with PSC.

Kaplan–Meier analysis of cumulative survival after liver transplantation from 01/2003 to 08/2007 at Hannover Medical School comparing 55 patients with PSC (incl. five patients with CC) and 318 patients with other chronic liver diseases (hepatitis B and C, alcoholic liver disease, hepatocellular carcinoma and others). The log-rank-test shows a significant better survival of patients with PSC (p<0.01).