Home > Diseases and Conditions > Primary biliary cirrhosis
Printer friendly viewPrint
Bookmark and Share

PubMed Health. A service of the National Library of Medicine, National Institutes of Health.

A.D.A.M. Medical Encyclopedia. Atlanta (GA): A.D.A.M.; 2011.

A.D.A.M. Medical Encyclopedia.

Primary biliary cirrhosis

PBC

Last reviewed: May 4, 2010.

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.

Causes, incidence, and risk factors

The cause of inflamed bile ducts in the liver is not known. 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

Tests for liver dysfunction:

  • Serum albumin

  • Liver function tests (serum alkaline phosphatase is most important)

  • Prothrombin time (PT)

  • Serum cholesterol and lipoproteins

Tests for the disease:

Treatment

Therapy aims to relieve symptoms and prevent complications.

Cholestyramine (or colestipol) may reduce the itching. Ursodeoxycholic acid may improve removal of bile from the bloodstream and improves the survival of patients with PBC after they 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

  1. Afdhal NH. Diseases of the gall bladder and bile ducts. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier. 2007: chap 159.
  2. Mayo MJ. Natural history of primary biliary cirrhosis. Clin Liver Dis. 2008;12:277-288.
  3. Silveira MG, Lindor KD. Treatment of primary biliary cirrhosis: therapy with choleretic and immunosuppressive agents. Clin Liver Dis. 2008;12:425-443.

Review Date: 5/4/2010.

Reviewed by: 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, CA. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is an independent audit to verify that A.D.A.M. follows rigorous standards of quality and accountability. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial policy, editorial process and privacy policy. A.D.A.M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only — they do not constitute endorsementscof those other sites. © 1997–2011 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

Copyright © 2012, A.D.A.M., Inc.

What works?

  • Bezafibrate for primary biliary cirrhosis Bezafibrate for primary biliary cirrhosis
    Primary biliary cirrhosis is a chronic disease of the liver that is characterised by progressive inflammation and destruction of the liver tissue, eventually progressing to liver cirrhosis and the need for liver transplantation. Primary biliary cirrhosis primarily affects middle‐aged women. Bezafibrate is a hypolipidaemic agent used in treatment of hypertriglyceridaemia. There are studies suggesting that bezafibrate, alone or in combination with ursodeoxycholic acid, is effective in treatment of primary biliary cirrhosis. Mechanisms through which bezafibrate improves lipid serum concentration balance and prevents biliary cell damage still need to be fully understood. This review evaluates all data on the benefits and harms of bezafibrate for patients with primary biliary cirrhosis in randomised clinical trials. The findings of this review are based on six randomised clinical trials with 151 Japanese patients. Bezafibrate was compared with no intervention in four trials (with co‐intervention of ursodeoxycholic acid in both the bezafibrate and control groups) and with ursodeoxycholic acid in two trials. The primary findings of the review are that bezafibrate has no statistically significant effects on mortality, liver‐related morbidity, adverse events, and quality of life of patients with primary biliary cirrhosis. A possible positive intervention effect of bezafibrate versus no intervention on liver biochemistry measures can be real but could also be due to systematic errors or random errors. The benefits and harms of bezafibrate for patients with primary biliary cirrhosis need further assessment in randomised clinical trials comparing bezafibrate with placebo. Such trials ought to be conducted with impeccable methodology to reduce the risks of random errors and sufficiently large patient groups to reduce the risks of random errors.
See all (14)...

Figures

  • Digestive system.
    Bile pathway.

Learn about...

  • How we know if a treatment works How we know if a treatment works
    Testing Treatments is a lively look at modern clinical research, and how everyone can get involved in using and improving research for better health care.

Drug of interest

  • Colestipol
    Colestipol is used with diet changes (restriction of cholesterol and fat intake) to reduce the amount of cholesterol and certain fatty substances in your blood. Accumulation of cholesterol and fats along the walls of your arteries (a process known as atherosclerosis) decreases blood flow and, therefore, the oxygen supply to your heart, brain, and other parts of your body. Lowering your blood level of cholesterol and fats may help to prevent heart disease, angina (chest pain), strokes, and heart attacks. This medication is sometimes prescribed for other uses; ask your doctor or pharmacist for more information.

Read More

MedlinePlus.gov links to free, reliable, up-to-date health information from the National Institutes of Health (NIH) and other trusted health organizations.

Recent activity

Clear Turn Off Turn On

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

See more...
You are here: NCBI > Literature > PubMed Health
Write to the Help Desk