Primary Biliary Cirrhosis "PBC"

This is te type of Cirrhosis Mom has.

Primary biliary cirrhosis is a disease in which the bile ducts in your liver are slowly destroyed. Your body has an intricate system of ducts designed specifically to transport bile, a fluid produced in your liver. Bile is essential for the proper digestion of fats and helps rid your body of worn-out red blood cells, cholesterol and potentially toxic metals. In primary biliary cirrhosis, the destruction of your bile ducts can cause harmful substances to build up in your liver and sometimes lead to irreversible scarring of liver tissue (cirrhosis).

The cause of primary biliary cirrhosis remains unclear. Many experts consider primary biliary cirrhosis an autoimmune disease in which the body turns against its own cells, although it's likely that genetic and environmental factors also play a part. Primary biliary cirrhosis develops slowly. Medication can slow the progression of the disease, especially if treatment begins early.

Causes

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 may play a role in this disease.

An immune system disorder?
Most evidence suggests that primary biliary cirrhosis is an immune system disorder. The initial inflammation begins with T lymphocytes (T cells) — white blood cells that normally recognize and help defend against bacteria and fungi. T cells invade and destroy the epithelial cells lining the small bile ducts. The T cells also produce chemicals that stimulate the epithelial cells to secrete proteins that attract more T cells, thereby creating an ongoing cycle of damage.

This is a classic autoimmune scenario — a case in which the body's immune system turns against its own cells. But not everything about primary biliary cirrhosis is classic. For one thing, it rarely occurs in children, even though autoimmune disorders often begin in childhood. For another, unlike most autoimmune diseases, primary biliary cirrhosis doesn't usually respond to drugs that suppress the immune system. For these and other reasons, researchers suspect that other factors play a part in the disease, such as:

Genetics. Primary biliary cirrhosis isn't transmitted from parent to child and so isn't considered a hereditary disease. Yet because it seems to run in families, researchers suspect that some people may inherit certain immune system defects that make them more susceptible to the disorder. Other immune system genes may play a role in disease progression.

Risk factors

The following factors may increase the risk of primary biliary cirrhosis:

• Your sex. More than 90 percent of people with primary biliary cirrhosis are women. Immune system disorders in general affect far more women than men, but the reason for this isn't known.
• Your age. Most people diagnosed with primary biliary cirrhosis are 35 to 60 years old. Although older adults can develop the disease, it is rare in children.
• Family history. Primary biliary cirrhosis isn't hereditary, but having a family member with the disease increases your risk.