Navigate Up

Heart Center - A-Z Index

#
J
Q
X
Z

Print This Page

Primary biliary cirrhosis

Primary biliary cirrhosis is irritation and swelling (inflammation) of the bile ducts of the liver, which blocks the flow of bile . This obstruction damages liver cells and leads to scarring called cirrhosis.

Alternative Names

PBC

Causes, incidence, and risk factors

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 more commonly affects middle-aged women.

Long-term bile obstruction is believed to lead to liver cirrhosis . The disease may be associated with autoimmune disorders such as:

Symptoms

More than half of patients have no symptoms at the time of diagnosis. Symptoms usually come on gradually and may include:

Signs and tests

The doctor or nurse will perform 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 may improve survival in some patients who have taken it for 4 years.

Vitamin replacement therapy restores vitamins A, K, and D, which are lost in fatty stools. A calcium supplement should be added to prevent or treat soft, weakened bones (osteomalacia ).

Liver transplant before liver failure occurs may be successful.

Expectations (prognosis)

The outcome can vary. If the condition is not treated, most patients will need a liver transplant to prevent death from this condition. About a quarter of patients who've had the disease for 10 years will experience liver failure. Doctors can now use a statistical model to predict the best time to do the transplant.

Complications

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

Calling your health care provider

Call your health care provider if you have:

References

Afdhal NH. Diseases of the gallbladder and bile ducts. In:Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 158.

Mayo MJ. Natural history of primary biliary cirrhosis. Clin Liver Dis. 2008;12:277-288.

Updated: 5/1/2012

David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. George F. Longstreth, MD, Department of Gastroenterology, Kaiser Permanente Medical Care Program, San Diego, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.


©  UPMC | Affiliated with the University of Pittsburgh Schools of the Health Sciences
Supplemental content provided by A.D.A.M. Health Solutions. All rights reserved.

For help in finding a doctor or health service that suits your needs, call the UPMC Referral Service at 412-647-UPMC (8762) or 1-800-533-UPMC (8762). Select option 1.

UPMC is an equal opportunity employer. UPMC policy prohibits discrimination or harassment on the basis of race, color, religion, ancestry, national origin, age, sex, genetics, sexual orientation, marital status, familial status, disability, veteran status, or any other legally protected group status. Further, UPMC will continue to support and promote equal employment opportunity, human dignity, and racial, ethnic, and cultural diversity. This policy applies to admissions, employment, and access to and treatment in UPMC programs and activities. This commitment is made by UPMC in accordance with federal, state, and/or local laws and regulations.

Medical information made available on UPMC.com is not intended to be used as a substitute for professional medical advice, diagnosis, or treatment. You should not rely entirely on this information for your health care needs. Ask your own doctor or health care provider any specific medical questions that you have. Further, UPMC.com is not a tool to be used in the case of an emergency. If an emergency arises, you should seek appropriate emergency medical services.

For UPMC Mercy Patients: As a Catholic hospital, UPMC Mercy abides by the Ethical and Religious Directives for Catholic Health Care Services, as determined by the United States Conference of Catholic Bishops. As such, UPMC Mercy neither endorses nor provides medical practices and/or procedures that contradict the moral teachings of the Roman Catholic Church.

© UPMC
Pittsburgh, PA, USA UPMC.com