Primary Biliary Cirrhosis | Digestive Health Program | Loyola Medicine

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Primary Biliary Cirrhosis

Overview and Facts about Primary Biliary Cirrhosis

Primary biliary cirrhosis is a disease in which the liver’s bile ducts are slowly damaged over time. Bile is an important fluid made by the liver that helps with digestive health, including absorption of fat, vitamins and cholesterol. As the ducts that carry bile away from the liver break down, the liquid begins to build up in the liver, causing scarring (also known as cirrhosis).

Unfortunately, primary biliary cirrhosis is not curable and will eventually cause death unless you can receive a liver transplant. After being diagnosed, most people live for about another 10 years. There are four stages to the disease, with Stage 1 being the most innocuous and Stage 4 being the worst.

Symptoms and Signs of Primary Biliary Cirrhosis

Unfortunately, primary biliary cirrhosis does not have a lot of symptoms until the disease has really taken hold. You might even have it for years without realizing it. However, once the disease starts progressing, you’ll notice some of the following symptoms:

  • Dry eyes and mouth
  • Feeling tired
  • Having itchy skin
  • Losing weight unexpectedly
  • Pain in your joints, muscles or bones
  • Pain in your upper right abdomen
  • Swelling in your feet
  • Swelling in your spleen
  • Diarrhea
  • High cholesterol​
  • Hyperpigmentation
  • Jaundice
  • Loss of appetite
  • Nausea or vomiting
  • Weakened bones

Causes and Risk Factors of Primary Biliary Cirrhosis

Unfortunately, doctors do not yet know exactly what causes primary biliary cirrhosis. However, they do believe it's an autoimmune disease, in which the body starts attacking its own healthy cells. This is what causes damage to your bile ducts.

Females are far more likely to develop primary biliary cirrhosis than men, as they account for 90 percent of cases. Other risk factors include:

  • Being between 30 and 60 years old
  • Having a family member with the disease
  • Living in North America or Europe
  • Smoking

Tests and Diagnosis of Primary Biliary Cirrhosis

Often, your doctor will become concerned about your risk for primary biliary cirrhosis after an ordinary blood test reveals high cholesterol or abnormal liver enzyme levels. This might lead them to order more tests, which can include:

  • Antimitochondrial antibody tests to see if you have an autoimmune disorder
  • Liver biopsy to remove a small piece of the liver and examine it closer
  • X-rays, CT scans, ultrasounds or an MRI to get a more in-depth look at the liver

Treatment and Care for Primary Biliary Cirrhosis

Unfortunately, there’s no real treatment for primary biliary cirrhosis. To cure the disease, your only hope is a liver transplant.

However, there are plenty of ways your doctor can mitigate your symptoms and slow the disease’s progression. The first step is to prescribe ursodeoxycholic acid, ursodiol or obeticholic acid. These are medicines that help bile flow better from your liver.

Other medications, such as methotrexate and colchicine, can suppress your immune system to prevent it from attacking your liver.

For your symptoms, your doctor might recommend antihistamines to eliminate itching, artificial tears to prevent dry eyes and saliva substitutes to cure dry mouth.