Primary biliary cirrhosis: Difference between revisions

Jump to navigation Jump to search
Line 9: Line 9:


==Diagnosis==
==Diagnosis==
===Liver Biopsy===
Primary biliary cirrhosis is characterized by interlobular bile duct destruction.  [[Histopathologic]] findings of primary biliary cirrhosis include:<ref name=pmid10976014>{{cite journal | pmid = 10976014 | doi=10.1053/bega.2000.0103 | volume=14 | issue=4 | title=Destruction of bile ducts in primary biliary cirrhosis | year=2000 | month=August | author=Nakanuma Y, Tsuneyama K, Sasaki M, Harada K | journal=Baillieres Best Pract Res Clin Gastroenterol | pages=549–70}}</ref>
*Inflammation of the bile ducts, characterized by intraepithelial [[lymphocyte]]s, and
*Periductal [[epithelioid]] [[granulomata]].
A liver biopsy is necessary to determine the stage of disease.
===Other Diagnostic studies===
Abdominal [[medical ultrasonography|ultrasound]] or a [[CT scan]] is usually performed to rule out blockage to the bile ducts. Previously most suspected sufferers underwent a [[liver biopsy]], and - if uncertainty remained  - [[endoscopic retrograde cholangiopancreatography]] (ERCP, an [[endoscopy|endoscopic]] investigation of the [[bile duct]]).
Now most patients are diagnosed without invasive investigation since the combination of anti-mitochondrial antibodies (see below) and typical (cholestatic) liver function tests are considered diagnostic. However, a liver biopsy is necessary to determine the stage of disease.


'''MR images of a patient with primary biliary cirrhosis'''
'''MR images of a patient with primary biliary cirrhosis'''

Revision as of 19:54, 16 April 2013

For patient information page on this topic, click here.

Primary Biliary Cirrhosis Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Primary Biliary Cirrhosis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X Ray

CT

MRI

Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Primary biliary cirrhosis On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Primary biliary cirrhosis

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Primary biliary cirrhosis

CDC on Primary biliary cirrhosis

Primary biliary cirrhosis in the news

Blogs on Primary biliary cirrhosis

Directions to Hospitals Treating Primary biliary cirrhosis

Risk calculators and risk factors for Primary biliary cirrhosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-In-Chief: Cafer Zorkun, M.D., Ph.D. [2] Prashanth Saddala M.B.B.S

Synonyms and keywords: Chronic non-suppurative destructive cholangitis, PBC

Causes

Diagnosis

MR images of a patient with primary biliary cirrhosis

Therapy

There is no known cure, but medication may slow the progression so that a normal lifespan and quality of life may be attainable for many patients. Specific treatment for fatigue, which may be debilitating in some patients, is limited and currently undergoing trials.

  • Ursodeoxycholic acid (Ursodiol) is the most frequently used treatment. This helps reduce the cholestasis and improves blood test results (liver function tests). It has a minimal effect on symptoms and whether it improves prognosis is controversial.
  • To relieve itching caused by bile acids in circulation, which would normally be removed by the liver, cholestyramine (a bile acid sequestrant) may be prescribed to absorb bile acids in the gut and be eliminated, rather than re-enter the blood stream. Alternative agents include naltrexone and rifampicin.
  • To relieve fatigue associated with primary biliary cirrhosis, current studies indicate that Provigil (modafinil) may be effective without damaging the liver.[1] Though off-patent, the limiting factor in the use of modafinil in the U.S. is cost. The manufacturer, Cephalon, has made agreements with manufacturers of generic modafinil to provide payments in exchange for delaying their sale of modafinil.[2] The FTC has filed suit against Cephalon alleging anti-competitive behavior.[3]
  • Patients with PBC have poor lipid-dependent absorption of Vitamins A, D, E, K.[4] Appropriate supplementation is recommended when bilirubin is elevated[5]. Multivitamins (esp. Vitamin D) and calcium are also recommended.
  • Patients with PBC are at elevated risk of developing osteoporosis[6] and esophageal varices[7] as compared to the general population and others with liver disease. Screening and treatment of these complications is an important part of the management of PBC.

As in all liver diseases, excessive consumption of alcohol is contraindicated.

In advanced cases, a liver transplant, if successful, results in a favorable prognosis. After liver transplant, the recurrence rate may be as high as 18% at 5 years, and up to 30% at 10 years. There is no consensus on risk factors for recurrence of the disease [8]

Obeticholic acid is in phase III clinical trials for PBC.[9]

References

  1. Modafinil#Primary_biliary_cirrhosis
    Ian Gan S, de Jongh M, Kaplan MM (2009). "Modafinil in the treatment of debilitating fatigue in primary biliary cirrhosis: a clinical experience". Dig. Dis. Sci. 54 (10): 2242–6. doi:10.1007/s10620-008-0613-3. PMID 19082890. Unknown parameter |month= ignored (help)
    Kumagi T, Heathcote EJ (2008). "Primary biliary cirrhosis". Orphanet J Rare Dis. 3: 1. doi:10.1186/1750-1172-3-1. PMC 2266722. PMID 18215315. Ref 157 viz:
    Jones DE, Newton JL (2007). "An open study of modafinil for the treatment of daytime somnolence and fatigue in primary biliary cirrhosis". Aliment. Pharmacol. Ther. 25 (4): 471–6. doi:10.1111/j.1365-2036.2006.03223.x. PMID 17270003. Unknown parameter |month= ignored (help)
  2. Modafinil#Patent_protection_and_antitrust_litigation
    Carrier MA (2011). "Provigil: A Case Study of Anticompetitive Behavior" (PDF). Hastings Science & Technology Law Journal. 3 (2): 441–452.
  3. http://www.ftc.gov/os/caselist/0610182/080213complaint.pdf
  4. Bruce R. Bacon; John G. O'Grady (2006). Comprehensive clinical hepatology. Elsevier Health Sciences. pp. 283–. ISBN 978-0-323-03675-7. Retrieved 29 June 2010.
  5. Lindor, KD (2009 Jul). "Primary biliary cirrhosis". Hepatology (Baltimore, Md.). 50 (1): 291–308. doi:10.1002/hep.22906. PMID 19554543. Unknown parameter |coauthors= ignored (help); Check date values in: |date= (help)
  6. Collier, Jane (2002). "Guidelines on the management of osteoporosis associated with chronic liver disease". Gut. 50: i1–i9. PMC 1867644. PMID 11788576. |access-date= requires |url= (help)
  7. Ali, AH (2011 Aug). "Varices in early histological stage primary biliary cirrhosis". Journal of Clinical Gastroenterology. 45 (7): e66–71. doi:10.1097/MCG.0b013e3181f18c4e. PMID 20856137. Unknown parameter |coauthors= ignored (help); Check date values in: |date= (help)
  8. Medical care of the Liver Trasplant Patient, 3rd Edition published 2006, editied by Paul G. Killenberg, page 429
  9. http://www.genengnews.com/gen-news-highlights/dainippon-sumitomo-pays-intercept-15m-for-phase-iii-liver-disease-drug/81244901/

Sources

Medical

  • Online Mendelian Inheritance in Man (OMIM) 109720
  • M. Eric Gershwin, John M. Vierling, Michael P. Manns, eds. Liver Immunology. Philadelphia, Pa.: Hanley and Belfus, 2003. ISBN 1-56053-499-0. (State of the art; technical.)
  • Marshall M. Kaplan, and M. Eric Gershwin, "Primary Biliary Cirrhosis", New Engl. J. of Medicine, 353:1261-1273 September 22, 2005 Number 12 . Review article
  • Carlo Selmi, Ross L. Coppel, and M. Eric Gershwin, "Primary Biliary Cirrhosis", in Noel R. Rose, Ian R. Mackey, eds, The Autoimmune Diseases, 4th edition, Academic Press, 2006

General

  • Sanjiv Chopra. The Liver Book: A Comprehensive Guide to Diagnosis, Treatment, and Recovery, Atria, 2002, ISBN 0-7434-0585-4
  • Melissa Palmer. Dr. Melissa Palmer's Guide to Hepatitis and Liver Disease: What You Need to Know, Avery Publishing Group; Revised edition May 24, 2004, ISBN 1-58333-188-3. her webpage.
  • Howard J. Worman. The Liver Disorders Sourcebook, McGraw-Hill, 1999, ISBN 0-7373-0090-6.

See also

External Links

de:Primär biliäre Zirrhose sv:primär biliär cirrhos


Template:WikiDoc Sources