The bile ducts are tubes that move bile from the liver to the small intestine. Bile is a substance that helps with digestion. All of the bile ducts together are called the biliary tract.
When the bile ducts become swollen or inflamed, this blocks the flow of bile. These changes can lead to scarring of the liver called cirrhosis. This is called biliary cirrhosis. Advanced cirrhosis can lead to liver failure.
The cause of inflamed bile ducts in the liver is not known. However, primary biliary cirrhosis is an autoimmune disorder. That means your body's immune system mistakenly attacks healthy tissue. The disease may be linked to autoimmune disorders such as:
The disease most often affects middle-aged women.
More than one half of people have no symptoms at the time of diagnosis. Symptoms most often begin slowly. Early symptoms may include:
- Nausea and belly pain
- Fatigue and loss of energy
- Fatty deposits under the skin
- Fatty stools
- Poor appetite and weight loss
As liver function worsens, symptoms may include:
- Fluid buildup in the legs (edema) and in the abdomen (ascites)
- Yellow color in the skin, mucous membranes, or eyes (jaundice)
- Redness on the palms of the hands
- In men, impotence, shrinking of the testicles, and breast swelling
- Easy bruising and abnormal bleeding, most often from swollen veins in the digestive tract
- Confusion or problems thinking
- Pale or clay-colored stools
Exams and Tests
The health care provider will do a physical exam.
The following tests can check to see if your liver is working properly:
- Albumin blood test
- Liver function tests (serum alkaline phosphatase is most important)
- Prothrombin time (PT)
- Cholesterol and lipoprotein blood tests
Other tests that can help measure how severe liver disease may be include:
The goal of treatment is to ease symptoms and prevent complications.
Cholestyramine (or colestipol) may reduce the itching. Ursodeoxycholic acid may improve removal of bile from the bloodstream. This may improve survival in some people. A newer drug called obeticholic acid (Ocaliva) is also available.
Vitamin replacement therapy restores vitamins A, K, E and D, which are lost in fatty stools. A calcium supplement or other bone medicines may be added to prevent or treat weak or soft bones.
Long-term monitoring and treatment of liver failure is needed.
Liver transplant may be successful if it is done before liver failure occurs.
The outcome can vary. If the condition is not treated, most people will die without a liver transplant. About one quarter of people who have had the disease for 10 years will have liver failure. Doctors can now use a statistical model to predict the best time to do the transplant. Other diseases, such as hypothyroidism and anemia, can also develop.
Progressive cirrhosis can lead to liver failure. Complications can include:
Primary biliary cholangitis; PBC
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Fogel EL, Sherman S. Diseases of the gallbladder and bile ducts. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 146.
Lamps LW. Liver: non-neoplastic diseases. In: Goldblum JR, Lamps LW, McKenney JK, Myers JL, eds. Rosai and Ackerman's Surgical Pathology. 11th ed. Philadelphia, PA: Elsevier; 2018:chap 19.
Smith A, Baumgartner K, Bositis C. Cirrhosis: diagnosis and management. Am Fam Physician. 2019;100(12):759-770. PMID: 31845776 pubmed.ncbi.nlm.nih.gov/31845776/.
Review Date 4/30/2020
Updated by: Bradley J. Winston, MD, board certified in gastroenterology and hepatology, Washington, DC. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.