Primary biliary cholangitis
Autoimmune disease of the liver / From Wikipedia, the free encyclopedia
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.
Primary biliary cholangitis | |
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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 |
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]