Primary Biliary Cirrhosis Diagnosis

According to the Mayo Clinic, primary biliary cirrhosis is a disease in which the bile ducts in your liver are slowly attacked and eventually ruined. When your bile ducts are destroyed by primary biliary cirrhosis harmful substances are allowed to accumulate in your liver. This may lead to permanent scarring of liver tissue (cirrhosis). Medication may help delay the progression of the disease but in some cases liver transplantation may be the best option.

Causes

• Under normal conditions, almost a quart of bile travels from the liver to the intestine on a daily basis. Primary biliary cirrhosis kills the small ducts within the liver, slowing and sometimes blocking the regular flow of bile.

  The precise cause of primary biliary cirrhosis is not known. The Mayo Clinic says some experts believe primary biliary cirrhosis is an autoimmune disease in which the body turns against its own cells.

Risk Factors

• Women are far more likely than to suffer from primary biliary cirrhosis. In fact, the Mayo Clinic says more than 90 percent of people with the disease are female. The majority of people diagnosed with primary biliary cirrhosis are between the ages of 35 and 60. It's believed that both genetics and environmental factors may contribute to the development of the disease.

Symptoms

• While some patients do not notice symptoms for years after the initial diagnosis of primary biliary cirrhosis, others may suffer from fatigue, itching, dry eyes and dry mouth. As the destruction of bile duct and liver cells worsens, other signs may include jaundice (a yellowing of your skin and the whites of your eyes) or hyperpigmentation (a darkening of the skin). Some women with primary biliary cirrhosis report frequent urinary tract infections.

Diagnosis

• There are a variety of testing methods available to aid in the diagnosis of primary biliary cirrhosis. Liver function tests examine a blood sample to check the levels of enzymes that may reveal liver dysfunction in general and bile duct injury specifically. During a liver biopsy a small sample of liver tissue is removed and studied under a microscope to diagnose the disease. If the biopsy is positive it can also identify how far the disease has progressed (staging).

Treatment

• Treatment for primary biliary cirrhosis is centered on slowing down the advancement of the disease, avoiding complications and minimizing symptoms. Ursodeoxycholic acid (UDCA) may help by impacting the body's bile acids and reducing inflammation. If started in the early stages of the disease, UDCA may extend life. However, it is not considered to be as effective for patients with severe liver damage.

Prognosis

• The prognosis for primary biliary cirrhosis is hard to predict. The National Institute of Health says that in most cases patients will need a liver transplant after approximately seven years.

  The NIH says a liver transplant is the only treatment available that will effectively cure primary biliary cirrhosis. Newer drugs that suppress the immune system have helped improve transplant survival rates. However, it's possible that the disease may return in the new liver.