Primary sclerosing cholangitis (PSC) and
primary biliary cholangitis (PBC) are immune-mediated chronic
liver diseases. PSC is a rare disorder characterized by multi-focal
bile duct strictures and progressive
liver diseases that ultimately results in the need for
liver transplantation in most
patients. Imaging studies, such as MRCP, have an essential
role in the
diagnosis of most cases of PSC. PSC is usually accompanied by
inflammatory bowel disease, and there is a high
risk of
cholangiocarcinoma and
colorectal cancer in PSC. No medical
therapies have been proven to delay the progression of PSC. Endoscopic intervention for
tissue diagnosis or biliary
drainage is frequently required in cases of PSC with a dominant
stricture, acute
cholangitis, or clinically suspected
cholangiocarcinoma. PBC is a chronic inflammatory autoimmune cholestatic
liver disease, which, when untreated,
will culminate in end-stage
biliary cirrhosis requiring
liver transplantation. A
diagnosis is usually based on the presence of
serum liver tests indicative of cholestatic
hepatitis in
association with circulating antimitochondrial
antibodies.
Patient presentation and
course can be diverse in PBC, and
risk stratification is important for ensuring that all
patients receive a personalized approach to their care. Medical
therapy using
ursodeoxycholic acid or obeticholic
acid has an important
role in reducing the progression to end-stage
liver disease in PBC.