24th July 2023

Primary biliary cholangitis (PBC)

Also known as primary biliary cirrhosis, PBC is an autoimmune condition which affects the bile ducts. It is also covered in greater detail in the Hepatic Pathology topic. Destruction of the epithelial cells of intrahepatic bile ducts and inflammation of the portal tract leads to progressive damage to the liver and biliary tree, which generally results in chronic, and then end-stage liver disease.

It is vastly more common in women than men, the most common age of onset is middle age, and it is associated with a spectrum of autoimmune diseases, especially Hashimoto’s thyroiditis, Sjogren’s syndrome and coeliac disease.

It most commonly presents with pruritus and fatigue, but many people are asymptomatic, with the condition diagnosed following investigation of incidental abnormal LFTs. As the disease progresses, it will manifest with the signs and symptoms of chronic liver disease and cirrhosis.

Typically, LFTs give a picture of elevated cholestatic markers (ALP/GGT). Antimitochondrial antibodies (AMA) or PBC-specific antinuclear antibodies (ANA) are further diagnostic indicators. Liver biopsy can be used to diagnose PBC, but as the serological markers are so accurate, biopsy and histology are generally not required for diagnosis.

Ursodeoxycholic acid is the first line treatment and aims to prevent disease progression. Cholestyramine is used for symptomatic relief of pruritus. Rifampicin or naltrexone can be used as second line agents.

Long term monitoring of the development of cirrhosis, and subsequent management, is also key.