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Causes
By Mayo Clinic staff
The exact cause of primary biliary cirrhosis isn't known, but it appears to be an immune system disorder that slowly destroys the bile ducts in your liver. Genetics and the environment also likely play a role in this disease.
Normally, bile is excreted into canal-like spaces between your liver cells, which drain into an interconnected series of thin tubes (ducts). The initial ducts are quite small, but become progressively larger as they spread through your liver, much like the branches of a tree.
Origin of the condition
The problems in primary biliary cirrhosis begin with inflammation in the smallest ducts in your liver. In time, the inflammation spreads to and destroys nearby liver cells. As these cells are destroyed, they're replaced by scar tissue (fibrosis). Over a period of years, the combination of ongoing inflammation, scarring and toxicity from trapped bile can lead to cirrhosis. Cirrhosis involves irreversible scarring of liver tissue that makes it impossible for your liver to carry out essential functions.
The inflammation begins when T lymphocytes (T cells) begin accumulating in your liver. T cells are white blood cells that are part of your immune system response. Normally, T cells recognize and help defend against bacteria and fungi. But in primary biliary cirrhosis, the T cells invade and destroy the cells lining the small bile ducts. The T cells also produce chemicals that stimulate liver cells to secrete proteins that attract more T cells, thereby creating an ongoing cycle of damage.
Researchers suspect that a genetic susceptibility coupled with an environmental trigger, such as infection, may be at the root of this abnormal immune response:
- Genetics. Primary biliary cirrhosis seems to run in families, and scientists believe that some people may inherit certain immune system defects that make them more susceptible to the disorder. Research has identified three genetic variations associated with primary biliary cirrhosis. This finding may eventually help researchers narrow in on the cause of primary biliary cirrhosis.
- Infection. For decades, researchers have suspected that primary biliary cirrhosis might result from a bacterial, fungal or parasitic infection, which would explain the massing of T cells in the small bile ducts. Some women reported having urinary tract infections, primarily those caused by the Escherichia coli bacterium, prior to the development of primary biliary cirrhosis. However, no commonplace infections have yet been consistently linked to primary biliary cirrhosis.
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- Picco MF (expert opinion). Mayo Clinic, Jacksonville, Fla. Aug. 31, 2009.
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