Primary biliary cirrhosis (PBC) is a disease in which the immune system damages the small bile ducts within the liver. Bile ducts are tiny tubes within the liver which drain bile into the gallbladder and intestine. Damage to the bile ducts causes bile to leak into the liver. This damages liver cells and leads to increasing scaring within the liver and eventually cirrhosis. This process takes approximately 20 years. Although the word “cirrhosis” is included in the name of this disease, the vast majority of patients with PBC do not have cirrhosis when the disease is first recognized.
PBC is not contagious. Patients inherit a genetic tendency to develop this disease and many persons with PBC have family members with other immune disorders. Patients with PBC frequently have other immune disorders as well. The most common of these is Rheumatoid Arthritis.
Many persons with PBC are asymptomatic. Of those persons with symptoms, the most common include fatigue, dry eyes, dry mouth and joint aches.
PBC causes the blood liver enzyme tests to be abnormal. A specific antibody is also present in the blood of patients with PBC. Patients with PBC also tend to have elevated blood cholesterol. However, the form of cholesterol that is elevated does not increase the risk for heart disease and in most cases does not require treatment.
The treatment for PBC is medication called Ursodeoxychilic acid or URSO. URSO is a non-toxic bile. It does not damage liver cells and reduces the liver damage caused by human bile. Many large studies conducted in this and other countries have clearly demonstrated that treating patients with URSO reduces liver damage in patients with PBC, prevents patients from developing cirrhosis and reduces the need for liver transplant. Patients in whom the liver enzymes return to normal or near normal when treated with URSO do not develop cirrhosis and appear to have a normal life expectancy. URSO is a pill taken orally once or twice daily. It has almost no side effects. Less than 5% of patients develop diarrhea or itching.
Dr. Shiffman was involved in studies which demonstrated that URSO was an effective treatment for PBC. Patients suspected of having PBC should undergo a complete evaluation by either Dr. Shiffman or April Long, NP at the Liver Institute of Virginia to determine if this is the correct diagnosis. Patients with PBC can also be receiving URSO can and also and should be monitored on a regular basis.