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Primary biliary cirrhosis

Digestive systemDigestive system
Bile pathwayBile pathway

Primary biliary cirrhosis; PBC

Definition

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.

Causes

The cause of inflamed bile ducts in the liver is not known. However, primary biliary cholangitis 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:

Celiac disease
Raynaud phenomenon
Sicca syndrome (dry eyes or mouth)
Thyroid disease

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 measure how severe liver disease may be include:

Elevated immunoglobulin M level in the blood
Liver biopsy
Anti-mitochondrial antibodies (results are positive in about 95% of cases)
Special types of ultrasound or MRI that measure the amount of scar tissue (may be called elastography)
Magnetic resonance cholangiopancreatography (MRCP)

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:

Bleeding
Damage to the brain (encephalopathy)
Fluid and electrolyte imbalance
Kidney failure
Malabsorption
Malnutrition
Soft or weak bones (osteomalacia or osteoporosis)
Ascites (fluid buildup in the abdominal cavity)
Increased risk of liver cancer

When to Contact a Medical Professional

Contact 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 cholangitis. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 11th ed. Philadelphia, PA: Elsevier; 2021:chap 91.

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/.

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Review Date: 5/4/2022

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

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