These conditions are disease of the bile ducts of the liver. Bile ducts normally are smooth and tapered and drain bile acids out of the liver into the small bowel. When these bile ducts become scarred and irregular in shape, bile acids do not drain normally and lead to liver damage. Primary biliary cholangitis involves the smaller bile ducts while primary sclerosing cholangitis involves larger bile ducts.

Primary biliary cholangitis (PBC) is more common in women than men. It is thought to be an immune mediated injury of the liver, but the exact cause is not known. Primary sclerosing cholangitis (PSC) is a disease involving larger bile ducts, and leads to cirrhosis in many people. It is often associated with ulcerative colitis and can occur in both men and women.

In early disease, neither condition has symptoms. PBC often leads to fatigue, itchy skin, dry eyes, dry mouth, fatty deposits of the skin around the eyes, and darkening of the skin. PSC is often associated with recurrent infections, itchy skin, and is often associated with inflammatory bowel disease. If not treated, both conditions lead to cirrhosis and liver failure. PSC is also associated with bile duct cancer.

Blood tests and liver biopsy are usually needed to diagnose PBC, while imaging is needed to diagnose PSC. Elevated alkaline phosphatase is the typical liver enzyme elevation noted on blood tests and this should lead to imaging. This can be done with ERCP or MRI scans for PSC. A high index of suspicion is needed to make the diagnosis in early disease.

It is important to diagnose biliary conditions early to prevent long-term liver damage. Once diagnosed, PBC is usually treated with a bile acid called ursodeoxycholic acid. PSC does not have an accepted therapy but requires close monitory. Both conditions sometimes require liver transplantation if they progress to cirrhosis.

Biliary conditions such as PSC or PBC are not common, but important to diagnose and monitor to prevent complications.