Say What?

Let’s say that you are a female around 35 years of age. Over the past several months, you have experienced unexplained fatigue and general body itching (pruritus). Finally, you go to see your family doctor, who tells you that you need to have some blood tests and an abdominal ultrasound. Once the results are available, your physician explains that you likely have primary biliary cirrhosis (PBC) and need to see a liver specialist. Your reaction is, “Say what?”

PBC is a chronic disease that causes the bile ducts in the liver to become inflamed and damaged and, ultimately, to disappear. Bile is a liquid produced in the liver that travels through the bile ducts to the gallbladder and then into the small intestine, where it helps digest fats and fat-soluble vitamins. When chronic inflammation damages the bile ducts, bile builds up in the liver, injuring liver tissue and, ultimately, leading to advanced scarring of the liver (cirrhosis).1

The exact cause of PBC is unknown but many experts consider it an autoimmune disease in which the body turns against its own cells. There may also be a genetic factor that makes a person more prone to developing PBC as well as other autoimmune diseases such as rheumatoid arthritis (affecting the joints) and Crohn’s disease (attacking the digestive tract). Some researchers believe that a bacterial, fungal, or parasitic infection could trigger PBC.2

Women are ten times more likely to develop PBC than are men. Physicians consider various aspects when making a PBC diagnosis. Blood tests will often show elevated liver enzymes, especially the alkaline phosphatase (ALP). As well, the test for anti-mitochondrial antibodies (AMA) is usually positive. Mitochondria are the energy sources within cells. Cholesterol levels may also be quite elevated but, interestingly, do not increase the risk of heart disease in persons with PBC. An abdominal ultrasound shows whether the liver and bile ducts are inflamed. A liver biopsy (a procedure in which a physician uses a needle to remove a small piece of the liver and then examine it under a microscope) will confirm the diagnosis and determine the extent of damage.3

Early in the disease, many patients have no symptoms. As mentioned earlier, fatigue and pruritus are the most common initial symptoms. As PBC progresses, other symptoms occur. There may be yellowing of skin and eyes from excess bile in the blood (jaundice), cholesterol deposits in the skin, fluid accumulation or edema, and darkening of the skin. Other immune related problems may also be present. For example, the tear and salivary glands may not function properly, causing dry eyes and mouth. Arthritis and thyroid problems may be present, and osteoporosis can develop in later stages, as the bones become soft and fragile, leading to an increased risk of fractures. The development of cirrhosis in the liver is the end result of PBC.4

Since PBC is incurable, physicians aim treatment at slowing progression of the disease, relieving symptoms, and preventing complications. Ursodeoxycholic acid (UDCA), a bile acid, helps move bile through the liver and is one of the most common medications used to treat PBC. This illness has numerous associations with other autoimmune disorders that may affect virtually any organ in the body, and your physician will manage these as they arise. Physicians treat other complications of PBC, such as osteoporosis, vitamin deficiencies, and cirrhosis, on an individual basis. They consider a liver transplant when they cannot control complications through treatment.3

Primary biliary cirrhosis is a slow, progressive disease. Once diagnosed, treatment can help to prolong survival. There is a great deal of research underway aimed at discovering the cause of PBC, preventing damage to the bile ducts and liver, improving symptoms, and prolonging life. By working closely with physicians, there is good reason for patients to expect a favourable long-term outlook.4

Lori Lee Walston, RN
First published in the Inside Tract® newsletter issue 181
1. National Digestive Diseases. Primary Biliary Cirrhosis. Available at: Accessed 2011-12-28.
2. Mayo Clinic. Primary Biliary Cirrhosis. Available at: Accessed 2011-12-28.
3. Palmer M. Dr. Melissa Palmer’s Guide to Hepatitis & Liver Disease. New York, NY: Avery, 2004. ISBN1-58333-188-3.
4. Jackson/Siegelbaum Gastroenterology. Primary Biliary Cirrhosis. Available at: Accessed 2011-12-28.