Large-duct cholangiopathies: aetiology, diagnosis and treatment

Abstract

Cholangiopathies describe a group of conditions affecting the intrahepatic and extrahepatic biliary tree. Impairment to bile flow and chronic cholestasis cause biliary inflammation, which leads to more permanent damage such as destruction of the small bile ducts (ductopaenia) and biliary cirrhosis. Most cholangiopathies are progressive and cause end-stage liver disease unless the physical obstruction to biliary flow can be reversed. This review considers large-duct cholangiopathies, such as primary sclerosing cholangitis, ischaemic cholangiopathy, portal biliopathy, recurrent pyogenic cholangitis and Caroli disease.

Keywords: biliary cirrhosis; biliary strictures; endoscopic retrograde pancreatography; portal hypertension; primary sclerosing cholangitis.

Figure 1

Histological features of primary sclerosing cholangitis (PSC). Characteristic concentric ‘onion-ring’ fibrosis and thickening of a bile ductule (*) is noted. The hepatic artery is indicated by the arrow.

Figure 2

Typical cholangiographic appearances of the biliary tree in primary sclerosing cholangitis with multifocal stricturing, sacculation and dilatation of the intrahepatic bile ducts (white arrow) and a dominant stricture in the common bile duct (black arrow).

Figure 3

Evolution of ischaemic biliary stricturing postliver transplant. (A) A biliary leak at the site of an early anastomotic stricture (arrow) was treated with stenting. (B) One month later, after the stent was removed, the patient had developed perihilar and peripheral classic intrahepatic beading and stricturing (white arrows), typical of an ischaemic cholangiopathy. The hepatic artery remained patent. The black arrow indicates the common bile duct.

Figure 4

Management algorithm for extrahepatic portal vein occlusion (EHPVO). ALP, alkaline phosphatase; EUS, endoscopic ultrasound; MRCP, magnetic resonance cholangiopancreatography.