Definition

What is primary biliary cirrhosis?

Bile, a fluid produced in your liver, plays a role in digesting food and helps rid your body of worn-out red blood cells, cholesterol and toxins. Primary biliary cirrhosis, sometimes called PBC, is a disease in which the bile ducts in your liver are slowly destroyed.

When bile ducts are damaged, as in primary biliary cirrhosis, harmful substances can build up in your liver and sometimes lead to irreversible scarring of liver tissue (cirrhosis).

How common is primary biliary cirrhosis?

Primary biliary cirrhosis can affect patients at any age. It can be managed by reducing your risk factors. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of primary biliary cirrhosis?

Although some people with primary biliary cirrhosis remain symptom-free for years after they’re diagnosed, others experience a number of signs and symptoms.

Common early symptoms include:

  • Fatigue
  • Itchy skin
  • Dry eyes and mouth

Later signs and symptoms may include:

  • Pain in the upper right portion of the abdomen
  • Bone, muscle or joint (musculoskeletal) pain
  • Yellowing of the skin and eyes (jaundice)
  • Darkening of the skin that’s not related to sun exposure (hyperpigmentation)
  • Swollen feet and ankles (edema)
  • Buildup of fluid in the abdomen due to liver failure (ascites)
  • Fatty deposits on the skin around your eyes, your eyelids, or in the creases in your palms, soles, elbows or knees (xanthomas)
  • Weak and brittle bones (osteoporosis), which can lead to fractures
  • Elevated cholesterol
  • Diarrhea that can be greasy (steatorrhea)
  • Underactive thyroid (hypothyroidism)

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes primary biliary cirrhosis?

It’s not clear what causes primary biliary cirrhosis. Many experts consider primary biliary cirrhosis an autoimmune disease in which the body turns against its own cells.

Risk factors

What increases my risk for primary biliary cirrhosis?

There are many risk factors for primary biliary cirrhosis, such as:

  • Your sex. The great majority of people with primary biliary cirrhosis are women.
  • Your age. Primary biliary cirrhosis is most likely to occur in people 30 to 60 years old.
  • Genetic factors. Having a family member with the disease makes it more likely that you will develop it.

Researchers think that genetic factors, when combined with environmental factors, trigger primary biliary cirrhosis. These environmental factors may include:

  • Researchers suspect primary biliary cirrhosis could be triggered by a bacterial, fungal or parasitic infection.
  • Smoking is associated with increased risk of primary biliary cirrhosis.
  • Environmental toxins. Some research suggests that toxic chemicals may play a role in developing primary biliary cirrhosis.

Diagnosis & Treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

 

How is primary biliary cirrhosis diagnosed?

Your doctor will first ask you about your health history and your family’s health history, and perform a physical exam.

The following tests and procedures may be used to diagnose primary biliary cirrhosis.

Laboratory tests:

  • Blood tests to check liver function. Liver function tests check the levels of enzymes that may indicate liver disease in general and bile duct injury in particular.
  • Blood tests to check for signs of autoimmune disease. An analysis of your blood may reveal anti-mitochondrial antibodies (AMAs). These antibodies almost never occur in people who don’t have the disease, even if they have other liver disorders. Therefore, a positive AMA test is considered a very reliable indicator of the disease. However, a small percentage of people with primary biliary cirrhosis don’t have AMAs.

Imaging tests:

  • Ultrasound uses high-frequency sound waves to produce images of structures inside your body.
  • Computerized tomography (CT scan). You may have a CT scan — a special X-ray technique that provides much more information than a standard X-ray does.
  • Magnetic resonance imaging (MRI). An MRI scanner uses a magnetic field and radio waves to create detailed images of organs and tissues. Unlike CT, there is no radiation exposure with MRI.
  • Magnetic resonance elastography (MRE). This newer test combines MRI imaging with sound waves to create a visual map (elastogram) of internal organs. The test is used to detect hardening of your liver that might indicate cirrhosis, similar to the way a doctor would examine (palpate) your body.
  • X-rays of your bile ducts. This test is invasive and may result in complications. With advances in MRI, it is usually not needed for diagnosis.

If the diagnosis is still uncertain, doctors may perform a liver biopsy. A small sample of liver tissue is removed and examined in a laboratory, either to confirm the diagnosis or to determine the extent (stage) of the disease.

How is primary biliary cirrhosis treated?

Because no cure exists for primary biliary cirrhosis, treatment focuses on slowing the progress of the disease, relieving symptoms and preventing complications.

Treatments aimed at slowing the disease and prolonging life include:

  • Ursodeoxycholic acid (UDCA). Also known as ursodiol (Actigall, Urso), UDCA is a bile acid that helps move bile through your liver. UDCA doesn’t cure primary biliary cirrhosis, but it may prolong life if started early in the disease and is commonly considered the first line of therapy.Although UDCA doesn’t work for everyone with primary biliary cirrhosis, people with early-stage disease respond better. UDCA is less likely to help people with advanced liver damage.
  • Liver transplant. When treatments no longer control primary biliary cirrhosis and the liver begins to fail, a liver transplant may help prolong life. A liver transplant is a procedure to remove your diseased liver and replace it with a healthy liver from a donor. Most donated livers come from people who have died. But in some cases, it may be possible to remove a portion of a liver from a living donor. Primary biliary cirrhosis often recurs in the transplanted liver, but it may take several years to develop.

Your doctor may recommend treatments to control the signs and symptoms of primary biliary cirrhosis to make you more comfortable. Treatments may help control the most common symptoms.

Working together, you and your doctor can help prevent some of the complications that can occur with primary biliary cirrhosis:

  • Increased pressure in the portal vein (portal hypertension). Your doctor is likely to screen for portal hypertension and enlarged veins when you’re first diagnosed and every few years thereafter. If you’re diagnosed with portal hypertension or bleeding, treatment may involve medications, such as a beta blocker or nitrate, or surgery.
  • Weak bones (osteoporosis). Treatment for bone loss associated with primary biliary cirrhosis often involves calcium and vitamin D supplements. Your doctor may also recommend that you exercise most days of the week to help increase your bone density.
  • Vitamin deficiencies. Your doctor may recommend supplements of vitamins A, D, E and K to counter vitamin deficiencies. Avoid taking herbs or nutritional supplements without talking to your doctor first.

Lifestyle changes & Home remedies

What are some lifestyle changes or home remedies that can help me manage primary biliary cirrhosis?

The following lifestyles and home remedies might help you cope with primary biliary cirrhosis:

  • Choose reduced-sodium foods. Opt for low-sodium foods or naturally sodium-free foods, since sodium contributes to tissue swelling and to the buildup of fluid in your abdominal cavity (ascites).
  • Exercise most days of the week. Exercise may reduce your risk of bone loss.
  • Avoid alcohol. Your liver processes the alcohol you drink, and the added stress can cause liver damage. Generally, people with primary biliary cirrhosis should abstain from alcohol.
  • Check with your doctor before starting new medications or dietary supplements. Because your liver isn’t working normally, you’ll likely be more sensitive to the effects of over-the-counter and prescription medications, as well as some dietary supplements, so check with your doctor before taking anything new.

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Review Date: March 9, 2017 | Last Modified: March 9, 2017

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