Primary Biliary Cholangitis

What is Primary Biliary Cholangitis?

Primary biliary cholangitis (PBC) is a progressive autoimmune disease of the liver. Over time, the small ducts that carry bile from the liver to the rest of the body become inflamed, gradually scarring and collapsing. As a result, bile and toxins can build up in the liver, leading to a condition called cholestasis. If untreated, cholestasis can lead to further damage to the liver, causing cirrhosis, an irreversible scarring of the liver, which may eventually lead to liver failure. PBC usually develops slowly and is more prevalent in middle-aged women.

Virginia Mason's Liver Center of Excellence in the Digestive Disease Institute is dedicated to progressive and innovative management of liver disorders, advanced liver disease such as cirrhosis and liver cancer.

To schedule an appointment with Virginia Mason's Liver Center specialists, call (206) 223-2319.

Symptoms

More than 50 percent of people with PBC do not have any noticeable symptoms when diagnosed. Common early symptoms include:

  • Dry eyes and mouth
  • Fatigue/sleepiness
  • Itchy skin

Advanced symptoms may include:

  • Ascites — build up of fluid in the abdomen
  • Darkening of the skin, unrelated to sun exposure
  • Diarrhea or greasy stools
  • Forgetfulness, mild confusion and irritability
  • Jaundice — yellowing of the skin or eyes
  • Pain in the upper-right abdomen, bones, muscles or joints
  • Swollen feet and ankles
  • Weight loss

Causes

Although the exact cause of PBC is unknown, it may be due to a combination of factors, including inherited genetics, environmental factors, and autoimmune response, when a person’s body mistakenly attacks its own healthy tissue. Unavoidable risk factors for PBC include:

  • Age: over 30 years
  • Genetics: PBC in family members, other autoimmune diseases
  • Geography: PBC is most common in northern Europe and North America
  • Sex: Women are 90 percent more likely than men to be affected

Diagnosis

Your provider may recommend tests or procedures to help identify primary biliary cholangitis:

  • Blood tests: A small amount of blood will be taken to assess alkaline phosphatase (ALP) and positive anti-mitochondrial antibody (+AMA). If these tests are elevated and positive, a diagnosis of PBC can be confirmed. Your provider may also test your blood for cholesterol and hypothyroidism, common conditions in the setting of PBC.
  • Screening for infections: Samples of your blood and urine will be tested for infection. If you have an infection, medications to treat it will be prescribed.
  • Examination of liver tissue:Your provider may recommend removing a small piece of liver tissue through a tiny incision on the abdomen or neck, a procedure called a liver biopsy, to determine if and to what extent PBC is present.

Treatment

Medical Therapy

Most patients respond well to medications prescribed by their provider to improve liver function, minimize symptoms like itching and fatigue, and prevent complications.

  • Ursodeoxycholic acid can reduce bile build-up and improve liver function.
  • Cholestyramine, stanozolol, naltrexone and rifampicin can reduce itching by absorbing bile acids and preventing their re-entry into the blood stream.
  • Modafinil may be prescribed for reducing fatigue.

Nutrition and Dietary Intake

  • Alcohol: Limit the amount of alcohol you drink to no more than one drink a day for women and two drinks a day for men.
  • Vitamins: Check with your provider regarding supplementing Vitamins A, D and E, which can be difficult to absorb in the setting of PBC. Avoid taking herbs or nutritional supplements without first discussing with your provider.

Liver Transplant

In very advanced cases of PBC, a liver transplant may be recommended.

To schedule an appointment with Virginia Mason's Liver Center specialists, call (206) 223-2319.