Sclerosing cholangitis refers to chronic cholestatic syndromes characterized by patchy inflammation, fibrosis, and strictures of the intrahepatic and extrahepatic bile ducts. Progression obliterates the bile ducts and leads to cirrhosis, liver failure, and sometimes cholangiocarcinoma.
(See also Overview of Biliary Function.)
Sclerosing cholangitis may be primary (with no known cause) or secondary due to immune deficiencies (congenital in children, acquired in adults as AIDS cholangiopathy), often associated with superimposed infections (eg, cytomegalovirus, Cryptosporidium), histiocytosis X, or use of drugs (eg, intraarterial floxuridine). Both primary and secondary sclerosing cholangitis cause similar inflammatory and fibrosing lesions scarring the bile ducts. Other causes of bile duct strictures are choledocholithiasis, postoperative biliary stricture, ischemic bile duct injury (during liver transplantation), congenital biliary abnormalities, cholangiocarcinoma, and parasitic infestations.
Diagnosis of biliary strictures and dilations requires imaging techniques such as ultrasonography and cholangiography.
Treatment focuses on relieving biliary obstruction (eg, dilating and stenting strictures) and, when possible, eradicating responsible organisms or treating the cause (eg, HIV).