Biliary Cirrhosis

Definition

Definition of Biliary Cirrhosis
Biliary cirrhosis, often abbreviated PBC, is an autoimmune disease of the liver marked by the slow progressive destruction of the small bile ducts (bile canaliculi) within the liver. When these ducts are damaged, bile builds up in the liver (cholestasis) and over time damages the tissue. This can lead to scarring, fibrosis and cirrhosis. It was previously thought to be a rare disease, but more recent studies have shown that it may affect up to 1 in 3-4,000 people; the sex ratio is at least 9:1 (female to male).

Symptoms

Symptoms of Biliary Cirrhosis
Many experts consider primary biliary cirrhosis an autoimmune disease in which the body turns against its own cells, although it's possible that genetic and environmental factors also play a part. Primary biliary cirrhosis develops somewhat slowly. Medication can slow the progression of the disease, especially if treatment starts early.

Causes

Causes of Biliary Cirrhosis
The exact cause of primary biliary cirrhosis is unknown, but it appears to be an immune system disorder that slowly destroys the bile ducts in the liver. Genetics and the environment may also play a role in this disease. Most evidence points out that primary biliary cirrhosis is an immune system disorder. The initial inflammation starts with T lymphocytes (T cells) - white blood cells that normally recognize and help defend against bacteria and fungi. T cells invade and kill the epithelial cells lining the small bile ducts. The T cells also give out chemicals that stimulate the epithelial cells to produce 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 fights against its own cells. Not everything, though, about primary biliary cirrhosis is classic. For one thing, it rarely occurs in young children, even though autoimmune disorders often begin in childhood. For another, unlike most autoimmune diseases, primary biliary cirrhosis does not generally respond to drugs that suppress the immune system.

Diagnosis

Diagnosis of Biliary Cirrhosis
The diagnosis of PBC should be based on a combination of historical, laboratory, serological and histological criteria. In general, patients are middle aged women who complain of pruritus early and jaundice late. Patients that present late in the course of their disease may also have symptoms of cirrhosis and hepatic failure. Many patients are referred for examination of an isolated elevated serum alkaline phosphatase activity on laboratory testing for other purposes. Essentially all patients have high serum alkaline phosphatase and gamma-glutamyltranspeptidase activities. The serum IgM concentration is almost always heightened.

Treatment

Treatment of Biliary Cirrhosis
Because no cure exists for primary biliary cirrhosis, treatment focuses mainly on slowing down the progress of the disease, relieving symptoms and preventing complications.

Prognosis

Prognosis of Biliary Cirrhosis
Consult with your doctor.

Prevention

Prevention of Biliary Cirrhosis
Consult with your doctor.