Other Names: PBC; Familial primary biliary cirrhosis; Primary Biliary Cirrhosis.

Primary biliary cholangitis (PBC) is a rare liver disease that affects the bile ducts within the liver and is caused by an autoimmune reaction. The autoimmune reaction damages bile ducts in the liver. Bile ducts are tube-like structures that carry bile from the liver to the intestine to help with the digestion of food. The bile ducts carry bile from the liver to the gallbladder, where it is stored. The bile ducts become inflamed and damaged, which causes bile to build up in the liver. This abnormal buildup destroys liver tissueand in later stages results in cirrhosis. Primary biliary cirrhosis usually occurs between the ages of 40 and 60 and affects women more often than men.

PBC is progressive, which means that the damage gets worse over time. Starting with inflammation, the damage can cause fibrosis, and then cirrhosis. In some cases, cirrhosis can lead to liver failure. The rate of damage caused by PBC varies in different people. If left untreated, PBC can get significantly worse in 2 years, making it important to get an early diagnosis to start treatment as quickly as possible.


Contact the Genetic and Rare Diseases (GARD) Information Center for more information on Primary Biliary Cholangitis.