What is Primary Biliary Cholangitis (PBC)?
PBC is a rare, chronic, autoimmune liver disease that progressively destroys the liver’s bile ducts. It mostly affects middle-aged women (10 women for every one man impacted), but it can be faster progressing and less responsive to therapy in men and younger women.1 Estimates indicate that PBC affects nearly 1 in 1,000 women over the age of 40.2
Bile ducts are tiny tubes that form the biliary tree, a network that collects and drains bile from the liver. Bile is a detergent-like substance composed of bile acid salts, cholesterol, and phospholipids. Bile collected from the liver is stored in the gall bladder and released into the small intestine in response to eating. In the intestine, its detergent-like properties combined with pancreatic juices help dissolve and digest food. In PBC, immune and inflammatory degeneration of bile ducts impairs the formation and flow of bile leading to the buildup of toxic bile acids.
PBC is progressive, with reinforcing cycles of cell death and regeneration, inflammation, and fibrosis (scarring). Complications include cirrhosis (excessive scarring), bleeding, accumulation of fluid in the abdomen, nervous system disorders, and sometimes liver failure. PBC has no cure, but medication can improve elevated liver enzymes and biomarkers that are associated with liver-related complications, liver transplantation, and death.