What causes primary biliary cirrhosis?
Primary biliary cirrhosis belongs to a group of disorders known as autoimmune diseases. In these diseases the body's immune system is fooled into attacking some of its own cells as though they were an infecting bacterium. In PBC the
immune system attacks the cells lining the bile ducts in the liver causing inflammation around them. Many patients also have similar reactions in their salivary or tear glands causing a dry mouth or an eye condition known as Sjögrens syndrome. It is thought that the immune system is misdirected due to a combination of genetic, hormonal and environmental factors.
Designed to protect the body from infection, the immune system of PBC patients attacks the liver causing slow, progressive damage to the bile ducts (small tubes through which bile flows from the liver to the gall bladder and intestines). When the bile ducts are damaged by inflammation, bile and other substances accumulate in the liver. The increased pressure causes further damage and eventually results in scarring of the liver (cirrhosis). Research has indicated that people with PBC display several abnormalities of the immune system, but what initially sparks off the bile duct damage is unknown. Generally PBC presents in women between thirty and fifty-five but it can also present in younger and older women and sometimes in men. About one in every ten cases are men. |
