Decrease (-) Restore Default Increase (+)
Bookmark and Share

Health Library

Back to MainBack to Main   Print This Page Print    Email to a Friend Email
 

Primary biliary cirrhosis

Definition

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

Alternative Names

PBC

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 more commonly affects middle-aged women.

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

Symptoms

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

Exams and Tests

The doctor or nurse 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 patients.

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 (osteoporosis, and rarely osteomalacia).

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 patients will die without a liver transplant. About a quarter of patients 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 health care provider if you have:

References

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

Angulo P, Lindor KD. Primary biliary cirrhosis. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger & Fordtran's Gastrointestinal and Liver Disease. 9th ed. Philadelphia, PA: Saunders Elsevier; 2010:chap 89.

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

Lindor KD, Gershwin ME, Poupon R, Kaplan M, et al. Primary biliary cirrhosis. American Association for Study of Liver Diseases. Hepatology. 2009;50:291.


Review Date: 5/15/2014
Reviewed By: Jenifer K. Lehrer, MD, Department of Gastroenterology, Frankford-Torresdale Hospital, Aria Health System, Philadelphia, PA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
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 endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
adam.com
 

Medical Care

Cancer
Pregnancy & Prenatal Classes
Weight Loss
Orthopedics
Heart Disease
Neurology
Women's Health
More Medical Care

Locations

Hospitals
Immediate Care
Health Centers
Emergency Room
Doctors Offices
Specialists
Affiliate Hospitals

Patients and Visitors

MyChart
Pay Your Bill
Request an Appointment
Get Healthy
Support Groups
Fitness Groups
Mobile Applications
Clinical Trials
Online Nursery
Classes and Events
Send an eCard
Patient Stories
Places to Stay
Say Thanks

About Us

Quality Report 
Careers
Ways to Help
Community Outreach
Contact Us
(502) 629-1234

Connect with us

© 2014 Norton Healthcare
Serving Kentucky and Southern Indiana