Primary Biliary Cirrhosis Treatment & Management

  • Author: Nikolaos T Pyrsopoulos, MD, PhD, MBA, FACP; Chief Editor: Julian Katz, MD   more...
 
Updated: Jan 4, 2012
 

Medical Care

The goals of treatment are to slow the progression rate of the disease and to alleviate the symptoms (eg, pruritus, osteoporosis, sicca syndrome). Liver transplantation appears to be the only life-saving procedure.

  • Ursodeoxycholic acid (UDCA) is the major medication used to slow the progression of the disease. Patients with early disease have clinical, biochemical, and histologic improvement. Reports suggest that UDCA delays the need for transplantation or delays death. The efficacy of this medication in late stages (ie, cirrhosis) is questionable. Patients who achieve biochemical response to UDCA after 1 year of treatment reportedly have a similar survival rate to the matched control population, and this observation might be used to identify the population of nonresponders who will require alternative or additional treatments. The use of UDCA after transplantation with evidence of recurrence of the disease has been associated with biochemical response, although its role in delaying histologic progression needs further investigation.[9, 10]
  • Immunosuppressive agents inhibit immune reactions that mediate the progression of the disease.
  • Methotrexate: Results of various trials suggest improvement in biochemical and histologic findings after treatment.
  • Corticosteroids may alleviate symptoms and improve biochemical and histologic findings. Corticosteroid-induced osteoporosis is of great concern. The dangers of using bisphosphonates requires further study.[11]
  • Cyclosporine has some therapeutic potential.
  • Colchicine has been used to limited effect.
  • Antipruritic treatment
    • Pruritus is often refractory to medical therapy and significantly impacts patients' quality of life. Antihistamines are first-line agents to relieve pruritus in early stages and are the first line of medication for patients with mild-to-moderate pruritus. Use caution in patients with cirrhosis and signs of encephalopathy because antihistamines can further depress brain function.
    • Cholestyramine and colestipol are effective in sequestering bile salts in the enteric lumen. A 1- to 4-day delay is expected before the itching remits.
    • Rifampin can also be used, but the precise mechanism of action is unclear (may involve inhibition of bile acid uptake into hepatocytes and facilitation of excretion of dihydroxy and monohydroxy bile acids and toxic bile acids). Rifampin is used in patients whose conditions are not responding to cholestyramine.
    • Some evidence suggests that dronabinol (Marinol) can be used to good effect.
    • Plasmapheresis has also been implemented for patients with severe pruritus intractable to medical treatment. Results have been good.
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Surgical Care

As the disease progresses to cirrhosis, an elevated bilirubin level, a prolonged prothrombin time, and a decreased albumin level can be found. The increased bilirubin level is an ominous sign of disease progression, and liver transplantation must be considered. Liver transplantation appears to be the only life-saving procedure.

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Activity

Increased activity is recommended, especially in postmenopausal women, to prevent osteoporosis.

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Contributor Information and Disclosures
Author

Nikolaos T Pyrsopoulos, MD, PhD, MBA, FACP  Chair of Hepatology, Medical Director of Liver Transplantation, Liver Unit, Florida Hospital; Associate Professor of Medicine, University of Central Florida College of Medicine

Nikolaos T Pyrsopoulos, MD, PhD, MBA, FACP is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Liver Foundation, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Transplantation, International Liver Transplantation Society, and Transplantation Society

Disclosure: Gilead Sciences Honoraria Speaking and teaching; Schering-Plough Honoraria Speaking and teaching; Roche Honoraria Speaking and teaching

Coauthor(s)

K Rajender Reddy, MD, FACP, FACG  Professor of Medicine, Director of Hepatology, Medical Director of Liver Transplantation, Hospital of the University of Pennsylvania

K Rajender Reddy, MD, FACP, FACG is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy, and Florida Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

George Y Wu, MD, PhD  Professor, Department of Medicine, Director, Hepatology Section, Herman Lopata Chair in Hepatitis Research, University of Connecticut School of Medicine

George Y Wu, MD, PhD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, American Medical Association, American Society for Clinical Investigation, and Association of American Physicians

Disclosure: Springer Consulting fee Consulting; Gilead Consulting fee Review panel membership; Gilead Honoraria Speaking and teaching; Bristol-Myers Squibb Honoraria Speaking and teaching; Springer Royalty Review panel membership

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

James L Achord, MD  Professor Emeritus, Department of Medicine, Division of Digestive Diseases, University of Mississippi School of Medicine

James L Achord, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Medical Association, American Society for Gastrointestinal Endoscopy, Mississippi State Medical Association, New York Academy of Sciences, Sigma Xi, and Southern Medical Association

Disclosure: Nothing to disclose.

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD  Clinical Professor of Medicine, Drexel University College of Medicine

Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law, Medicine & Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility

Disclosure: Nothing to disclose.

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This histologic picture is compatible with stage 2 primary biliary cirrhosis.
 
 
 
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