Cholestatic liver disease
What is Cholestatic liver disease?
The condition known by its abbreviation PBC, standing for primary biliary cirrhosis, is an autoimmune disease of the liver. It is marked by slow progressive destruction of the small bile ducts of the liver, with the intralobular ducts (Canals of Hering) affected early in the disease. When these ducts are damaged, bile and other toxins build up in the liver (cholestasis) and over time damages the liver tissue in combination with ongoing immune related damage. This can lead to scarring, fibrosis and cirrhosis. As cirrhosis is only a feature of advanced disease, a change of name to primary biliary cholangitis was proposed in 2014. This retains the initials of PBC but reflects more accurately the nature of the disease. It has not yet (April 2015) been adopted in scientific publications.
PBC was previously thought to be a rare disease, but more recent studies have shown that it may affect up to 1 in 3–4,000 people; the sex ratio is at least 9:1 female to male.