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Primary biliary cirrhosis

Primary biliary cholangitis; PBC

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. The buildup of bile damages the liver cells and leads to scarring of the liver called cirrhosis. This is called biliary cirrhosis.

Causes

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.

Symptoms

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
  • Itching
  • 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 diagnose this disease are:

Treatment

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.

Outlook (Prognosis)

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.

Possible Complications

Progressive cirrhosis can lead to liver failure. Complications can include:

When to Contact a Medical Professional

Call your provider if you have:

  • Abdominal swelling
  • Blood in the stools
  • Confusion
  • Jaundice
  • Itching of the skin that does not go away and is not related to other causes
  • Vomiting blood

References

Eaton JE, Lindor KD. Primary biliary cirrhosis. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 10th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 91.

Fogel EL, Sherman S. Diseases of the gallbladder and bile ducts. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 155.

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.

  • Digestive system - illustration

    The esophagus, stomach, large and small intestine, aided by the liver, gallbladder and pancreas convert the nutritive components of food into energy and break down the non-nutritive components into waste to be excreted.

    Digestive system

    illustration

  • Bile pathway - illustration

    The biliary system is comprised of the organs and duct system that create, transport, store and release bile into the duodenum for digestion. Includes the liver, gallbladder and bile ducts (named the cystic, hepatic, common, and pancreatic duct).

    Bile pathway

    illustration

  • Digestive system - illustration

    The esophagus, stomach, large and small intestine, aided by the liver, gallbladder and pancreas convert the nutritive components of food into energy and break down the non-nutritive components into waste to be excreted.

    Digestive system

    illustration

  • Bile pathway - illustration

    The biliary system is comprised of the organs and duct system that create, transport, store and release bile into the duodenum for digestion. Includes the liver, gallbladder and bile ducts (named the cystic, hepatic, common, and pancreatic duct).

    Bile pathway

    illustration

A Closer Look

 

Tests for Primary biliary cirrhosis

 
 

Review Date: 4/7/2018

Reviewed By: Michael M. Phillips, MD, Clinical Professor of Medicine, The George Washington University School of Medicine, Washington, DC. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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