Primary biliary cholangitis

Primary biliary cholangitis
Other names	Primary biliary cirrhosis
Micrograph of PBC showing bile duct inflammation and injury, H&E stain
Specialty	Gastroenterology, Hepatology
Symptoms	Cholestasis, pruritus, fatigue
Complications	Cirrhosis, hepatic failure, portal hypertension
Usual onset	Usually middle-aged women
Causes	Autoimmune
Risk factors	Female sex
Diagnostic method	Anti-mitochondrial antibodies, Liver biopsy
Differential diagnosis	Autoimmune hepatitis
Treatment	Ursodeoxycholic acid, obeticholic acid, cholestyramine
Frequency	1 in 3,000–4,000 people

Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune disease of the liver.^[1][2][3] It results from a slow, progressive destruction of the small bile ducts of the liver, causing bile and other toxins to build up in the liver, a condition called cholestasis. Further slow damage to the liver tissue can lead to scarring, fibrosis, and eventually cirrhosis.

Common symptoms are tiredness, itching, and in more advanced cases, jaundice. In early cases, the only changes may be those seen in blood tests.^[4]

PBC is a relatively rare disease, affecting up to one in 3,000–4,000 people.^[5][6] As with many other autoimmune diseases, it is much more common in women,^[7] with a sex ratio of at least 9:1 female to male.^[1] The reasons for this disparity are unclear, but may involve the expression of sex hormones such as estrogen, which impact immune system response.^[7]

The condition has been recognised since at least 1851, and was named "primary biliary cirrhosis" in 1949.^[8] Because cirrhosis is a feature only of advanced disease, a change of its name to "primary biliary cholangitis" was proposed by patient advocacy groups in 2014.^[9][10]