Primary biliary cirrhosis (pbc)

Abdomen | Gastroenterology | Primary biliary cirrhosis (pbc) (Disease)


Description

Primary 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).

Causes and Risk factors

The cause of the disease is unknown at this time, but research indicates that there is an immunological basis for the disease.

Diagnosis and Treatment

Blood tests will be done to determine the cause and severity of the liver damage.

The goal of treatment is to ease symptoms and prevent complications. Cholestyramine (or colestipol) may reduce the itching. Ursodeoxycholic acid may improve removal of bile from the bloodstream may improve survival in some patients who have taken it for 4 years.

Vitamin replacement therapy restores vitamins A, K, and D, which are lost in fatty stools. A calcium supplement should be added to prevent or treat soft, weakened bones (osteomalacia). Liver transplant before liver failure occurs may be successful. ...