Primary Biliary Cirrhosis

Overview

Mayo Clinic treats 500-600 patients who have primary biliary cirrhosis (PBC) each year. Liver specialists, oncologists, radiologists, pathologists and surgeons work together to provide comprehensive and coordinated care for their patients. These Mayo experts on PBC and related diseases have made many contributions to research on this disease.

PBC got its name because it was commonly diagnosed in its final stages when the liver was scarred, signifying cirrhosis. However, most patients have only mild scarring and do not have true cirrhosis. In the later stages of PBC, liver failure caused by scarring may warrant a liver transplant. PBC patients have the highest survival rate after liver transplant of all patient groups.

Diagnosis

Most often, PBC is diagnosed after a routine blood test or health evaluation turns up an abnormality. Many patients with PBC have no symptoms. Some possible symptoms include fatigue, jaundice and itching. Once the diagnosis is made, usually with a blood test, physicians can determine the stage of the disease with a liver biopsy. Read more about primary biliary cirrhosis diagnosis.

Treatment Options

A medication called ursodeoxycholic acid, can slow the progression of the disease in many patients. Other medications can help lessen symptoms of the disease. If medical treatment no longer controls PBC and severe liver failure occurs, a liver transplant may be necessary. Transplants can extend or improve the quality of life for people with PBC. Read more about primary biliary cirrhosis treatment options.

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About Primary Biliary Cirrhosis

PBC is a chronic disease that slowly destroys the liver. One liver function is to produce a digestive fluid called bile. Bile is created and drains into bile ducts that carry it into the intestines. For unknown reasons, sometimes these ducts become irritated and inflamed. As the inflammation spreads, the disease destroys the bile ducts and replaces them with scar tissue.

PBC is thought to be an autoimmune disease, where the body attacks its own cells. The cells attacked are those that line the bile ducts of the liver. The autoimmune response may be due to:

• Exposure to something in the environment
• A virus or bacteria
• Something in a patient's genetic makeup that makes him or her more susceptible

A number of diseases are associated with PBC, possibly because of problems with the body's immune response. Patients with PBC may be more likely to have the following conditions: osteoporosis, inflammatory arthritis, thyroid disorders or Sicca syndrome (a condition in which the tear glands along the eyelids and the salivary glands in the mouth do not produce enough moisture).