Definition

What is primary biliary cirrhosis?

Primary biliary cirrhosis, sometimes called PBC, is a disease in which the bile ducts in your liver are slowly destroyed. 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.

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).

Primary biliary cirrhosis is considered an autoimmune disease, in which the body turns against its own cells. Researchers think it is triggered by a combination of genetic and environmental factors. Primary biliary cirrhosis usually develops slowly and medication can slow its progression, especially if treatment begins early.

How common is primary biliary cirrhosis?

Please discuss with your doctor for further information.

Symptoms

What are the symptoms of primary biliary cirrhosis?

The common symptoms of primary biliary cirrhosis are:

  • Itching (pruritus).
  • Fatigue
  • Yellowing of the skin and the white part of the eyes (jaundice).
  • Discomfort in the upper right part of the abdomen.
  • Dry eyes and mouth.
  • Vaginal dryness.

With more advanced liver damage, people may have complications related to cholangitis such as:

  • Fluid buildup in the abdomen (ascites).
  • Bleeding of enlarged veins in the esophagus, stomach, and rectum (variceal bleeding).
  • Premature thinning of the bones (osteoporosis).

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.

How primary biliary cirrhosis develops

The inflammation of primary biliary cirrhosis begins when T lymphocytes (T cells) start accumulating in your liver. T cells are white blood cells that are part of your immune system response.

Normally, T cells recognize and help defend against harmful invaders, such as bacteria.  But in primary biliary cirrhosis, the T cells invade and destroy the cells lining the small bile ducts in your liver.

Inflammation in the smallest ducts spreads, in time, and destroys nearby liver cells. As these cells are destroyed, they’re replaced by scar tissue (fibrosis) that can contribute to cirrhosis. Cirrhosis is scarring of liver tissue that makes it difficult for your liver to carry out essential functions.

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 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?

PBC is suspected based on a medical history, physical exam, and lab tests that show high levels of the enzyme alkaline phosphatase and antimitochondrial antibodies (AMA) in the blood. Your doctor may also conduct an imaging test such as an ultrasound, which shows images of the liver and other tissues inside the body. He or she may also do a biopsy to remove a small piece of tissue from the liver. Biopsy is the best way to definitively diagnose PBC.

How is primary biliary cirrhosis treated?

Treatment for primary biliary cirrhosis is difficult, because the immune system appears to be involved. As with other forms of cirrhosis, treatment focuses on reducing symptoms, preventing and treating the complications of the disease, and preventing other conditions that may cause additional liver damage.

Two medicines, ursodiol (UDCA) and ocaliva, can be used to treat PBC. Both medicines help move bile out of the liver into the small intestine. And Ocaliva decreases the amount of bile acids made by the liver.

Medicines such as cholestyramine, rifampicin, or naltrexone may be used to help with itching caused by PBC.

If you have fatigue, your doctor will try to find the cause of your tiredness. It may not be caused by PBC. Some medicines have been tried to help with fatigue in PBC, but none have been proved to work well.

PBC can also cause dry eyes and mouth. The best way to help dry eyes is to use eye drops (artificial tears) when your eyes feel dry. A dry mouth can be helped by chewing gum or hard candy to increase saliva. You can also use a saliva substitute. If that doesn’t help, there are some medicines that can be used.

Because people with PBC have a high risk of getting osteoporosis, you should have periodic bone density scans. Your doctor may suggest that you take calcium and vitamin D supplements and perhaps a medicine called a bisphosphonate.

Liver transplantation may be the treatment of choice for people who have end-stage primary biliary cholangitis, although primary biliary cholangitis can recur after a liver transplant.

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.

 

Sources

Review Date: January 2, 2018 | Last Modified: January 2, 2018

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