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Primary Biliary Cholangitis

This month we are focusing on primary biliary cholangitis (PBC), an autoimmune disorder that attacks the liver. We asked John M. Vierling, MD, director of Baylor Liver Health and chief of hepatology at Baylor College of Medicine, about this disease, which occurs most commonly in individuals between the ages of 35 and 60. Women account for about 90 percent of PBC cases. Dr. Vierling is also a member of the American Liver Foundation’s national medical advisory committee, co-chair of its Houston office medical advisory committee and former chair of the American Liver Foundation’s board of directors.

What is primary biliary cholangitis?

Primary biliary cholangitis is a chronic autoimmune disease of the liver that slowly destroys its small to medium-sized bile ducts. This causes bile to remain in the liver, which can damage cells and cause scarring that can lead to cirrhosis if unrecognized and untreated.

Bile is a liquid that is made in the liver and travels down the bile ducts to enter the duodenum, the portion of the small bowel next to the stomach. Bile contains acids, which are needed to digest fats and absorb fat-soluble vitamins A, D, E and K. These acids also act as hormones with important effects in maintaining health.

PBC advances slowly, providing doctors an opportunity for early treatment. Many patients lead active and productive lives for more than 10 to 15 years after diagnosis. In fact, patients who show no symptoms at the time of diagnosis often remain symptom-free for years. And the good news is that patients whose liver tests return to normal on treatment can expect a normal life expectancy.

If cirrhosis develops, PBC may lead to life-threatening complications. Cirrhosis occurs when scar tissue blocks the flow of blood through the liver and impairs its ability to function normally.

If the liver cannot filter waste from the intestine, confusion and altered levels of consciousness (hepatic encephalopathy) can result.

However, the majority of PBC patients are diagnosed before progression to cirrhosis and current treatments can even prevent its development. So the word “cirrhosis” in the disease name is not really accurate. The word “cirrhosis” also carries negative connotations among the general public. A new global effort is underway to change the name of the disease to primary biliary cholangitis, which better describes the inflammatory nature of the disease.

The goals of therapy are to prevent development of cirrhosis in patients with earlier stages of disease and slow progression and prevent deterioration in patients who already have cirrhosis.

What are the signs and symptoms of PBC?

Many people with PBC do not have symptoms, especially in its early stages. When symptoms do occur, the most common is intense itching of the skin (pruritus), often in the arms, legs and back. Other symptoms may include fatigue, jaundice, fluid build-up in the ankles and abdomen, darkening of the skin and collection of fatty deposits in the skin around the eye. Jaundice is an indication of advanced disease and is absent in most patients when they are diagnosed.

Several other autoimmune disorders often occur with PBC. The most common are dry eyes and mouth (Sjogren syndrome), scleroderma, hypothyroidism, arthritis and kidney stones.

Another thing to note is that people with PBC also face increased risk of osteoporosis, which increases their risk of fractures in later stages. They also are at higher risk of developing gallstones.

What causes PBC?

The cause of PBC is not known. Although it is technically not a genetic disease, there is a family link. PBC is more common among siblings and in families where one member has been affected.

How is PBC diagnosed?

Because many PBC patients have no symptoms, the disease is often discovered through abnormal results on routine liver blood tests. Doctors need to do several tests to confirm a diagnosis. One test looks for the presence of antimitochondrial autoantibodies in the blood. This test is positive in nearly all PBC patients. Ultrasound exams and a liver biopsy, in which a small sample of liver tissue is removed with a needle, may also be performed.

How is PBC treated?

Patients most often respond to bile acid therapy taken by mouth daily for life. The medication has minimal side effects, improves the liver’s ability to function and retards progression of cirrhosis. Treatment has been shown to extend life expectancy in patients with or without cirrhosis, delaying or preventing the need for a liver transplant.

What lifestyle changes may be helpful for PBC patients?

Maintaining a healthy lifestyle helps patients feel better, prevents loss of calcium from bones and may relieve or prevent some of the secondary symptoms of PBC. Your doctor may give you recommendations about:

  • A reduced sodium diet
  • Drinking ample water
  • Taking calcium and vitamin D supplements
  • Avoiding or reducing the consumption of alcohol
  • Reducing stress
  • Exercising, particularly walking
  • Skin care
  • Regular dental examinations
  • Artificial tears for dry eyes

What is being done to find a cure for PBC?

PBC was discovered more than 150 years ago, but it was only in the last 40 years that doctors developed the ability to diagnose it very early and provide effective treatment. This means that treatment can begin before the liver is severely damaged. Scientists are continuing to study the disease to find the cause and understand its development.

In addition, investigators are exploring the use of several new medications to lessen the symptoms and control liver damage through drug therapy trials involving a large number of patients around the world.

Last Updated on May 20, 2020


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