It's not clear what causes primary biliary cholangitis. Many experts consider it an autoimmune disease in which the body turns against its own cells.
The liver inflammation seen in primary biliary cholangitis starts when certain types of white blood cells called T cells (T lymphocytes) start to collect in the liver. Normally, these immune cells detect and help defend against germs, such as bacteria. But in primary biliary cholangitis, they mistakenly destroy the healthy cells lining the small bile ducts in the liver.
Inflammation in the smallest ducts spreads and eventually damages other cells in the liver. As the cells die, they're replaced by scar tissue (fibrosis) that can lead to cirrhosis.
Risk factors:
The following factors may increase your risk of primary biliary cholangitis:
• Sex: Most people with primary biliary cholangitis are women.
• Age: It's most likely to occur in people 30 to 60 years old.
• Genetic factors: You're more likely to get the condition if you have a family member who has or had it.
Courtesy: Mayo Clinic