Primary biliary cirrhosis: Difference between revisions

Jump to navigation Jump to search
m (Bot: Removing from Primary care)
 
(40 intermediate revisions by 6 users not shown)
Line 1: Line 1:
{{Infobox_Disease |
__NOTOC__
  Name          = {{PAGENAME}} |
  Image          = |
  Caption        = |
  DiseasesDB    = 10615 |
  ICD10          = {{ICD10|K|74|3|k|70}} |
  ICD9          = {{ICD9|571.6}} |
  ICDO          = |
  OMIM          = |
  MedlinePlus    = |
  MeshID        = D008105 |
}}
{{Search infobox}}
{{CMG}} '''Associate Editor(s)-In-Chief:''' {{CZ}} [[User: Prashanthsaddala|Prashanth Saddala M.B.B.S]]


{{SK}} Chronic non-suppurative destructive cholangitis, PBC
'''For patient information page on this topic, click [[Primary biliary cirrhosis (patient information)|here]].'''
==Overview==
{{Primary biliary cirrhosis}}
'''Primary biliary cirrhosis''' is an [[autoimmune disease]] of the [[liver]] marked by the slow progressive destruction of the small bile ducts ([[bile canaliculi]]) within the liver. When these ducts are damaged [[bile]] builds up in the liver ([[cholestasis]]) and over time damages the tissue. This can lead to scarring, [[fibrosis]], [[cirrhosis]], and ultimately [[liver failure]].
==Historical Perspective==
Addison and Gull in 1851 described the clinical picture of progressive obstructive jaundice in the absence of mechanical obstruction of the large bile ducts. Ahrens ''et al'' in 1950 coined the term primary biliary cirrhosis for this disease. The association with anti mitochondrial antibodies was first reported in 1986.<ref name=Mitchison1986>{{cite journal |author=Mitchison HC, Bassendine MF, Hendrick A, ''et al.'' |title=Positive antimitochondrial antibody but normal alkaline phosphatase: is this primary biliary cirrhosis? |journal=Hepatology |volume=6 |issue=6 |pages=1279–84 |year=1986 |pmid=3793004 |doi=10.1002/hep.1840060609}}</ref>
==Pathophysiology==
===Histopathology===
{{#ev:youtube|Jj8ozr_IttM&mode=related&search=}}


==Causes==
{{CMG}}; {{AE}}{{Anmol}}, {{SH}}, {{AA}}, {{ARK}}
The cause of the disease is unknown at this time, but research indicates that there is an [[Immunology|immunological]] basis for the [[disease]], making it an [[autoimmune disorder]].  Most of the patients (>90%) seem to have anti-mitochondrial antibodies (AMAs) against [[pyruvate dehydrogenase complex]]  (PDC-E2), an enzyme complex that is found in the [[mitochondria]].


In addition, a more specific test to confirm this disease from a bone disorder such as Paget's (disease of bone) which also has increases in Alkaline phosphatase is the [[Gamma glutamyl transpeptidase|Gamma-glutamyl trans peptidase]] test (GGTP). An increase in GGTP could indicate presence of Primary Biliary Cirrhosis.
{{SK}} Chronic non-suppurative destructive cholangitis, PBC, Primary biliary cholangitis.


57% of patients with acute liver failure have [[anti-transglutaminase antibodies]]<ref name="pmid17657817">{{cite journal | author = Leung PS, Rossaro L, Davis PA, ''et al'' | title = Antimitochondrial antibodies in acute liver failure: Implications for primary biliary cirrhosis | journal = | volume = | issue = | pages = | year = 2007 | pmid = 17657817 | doi = 10.1002/hep.21828}}</ref>  suggesting a role of [[gluten sensitivity]] in primary biliary cirrhosis, and primary biliary cirrhosis is considerable more common in [[Gluten-sensitive enteropathy associated conditions#Diseases of the pancreas.2C gall bladder.2C bile duct|gluten sensitive enteropathy]] than the normal population.<ref name="pmid74661">{{cite journal | author = Logan RF, Ferguson A, Finlayson ND, Weir DG | title = Primary biliary cirrhosis and coeliac disease: an association? | journal = Lancet | volume = 1 | issue = 8058 | pages = 230-3 | year = 1978 | pmid = 74661 | doi = }}</ref><ref name="pmid12385447">{{cite journal | author = Volta U, Rodrigo L, Granito A, ''et al'' | title = Celiac disease in autoimmune cholestatic liver disorders | journal = Am. J. Gastroenterol. | volume = 97 | issue = 10 | pages = 2609-13 | year = 2002 | pmid = 12385447 | doi = }}</ref>
==[[Primary biliary cirrhosis overview|Overview]]==


In some cases of disease, protein expression may cause an [[immune tolerance]] failure, as might be the case with [[nucleoporin 210kDa| gp210]] and [[nucleoporin 62|p62]], nuclear pore proteins. Gp210 has increased expression in the bile duct of anti-gp210 positive patients.<ref name="pmid16337775">{{cite journal | author = Nakamura M, Takii Y, Ito M, ''et al'' | title = Increased expression of nuclear envelope gp210 antigen in small bile ducts in primary biliary cirrhosis | journal = J. Autoimmun. | volume = 26 | issue = 2 | pages = 138-45 | year = 2006 | pmid = 16337775 | doi = 10.1016/j.jaut.2005.10.007}}</ref> Both proteins appear to be prognostic of liver failure relative to anti-mitochondrial antibodies.
==[[Primary biliary cirrhosis historical perspective|Historical Perspective]]==


A genetic predisposition to disease has been thought important for some time, as evident by cases of PBC in family members, concordance in identical twins, and clustering of autoimmune diseases.  In 2009 a Canadian led group of investigators reported in the New England Journal of Medicine results from the first genome scan of PBC patients.<ref>{{cite journal |author=Hirschfield GM, Liu X, Xu C, ''et al.'' |title=Primary biliary cirrhosis associated with HLA, IL12A, and IL12RB2 variants |journal=N. Engl. J. Med. |volume=360 |issue=24 |pages=2544–55 |year=2009 |month=June |pmid=19458352 |pmc=2857316 |doi=10.1056/NEJMoa0810440 |url=http://content.nejm.org/cgi/content/full/NEJMoa0810440}}</ref><ref>http://www.torontoliver.ca</ref>  This research revealed parts of the IL12 signaling cascade, particularly IL12A and IL12RB2 polymorphisms, to be important in the etiology of the disease in addition to the HLA region, suggesting future therapeutic targets.
==[[Primary biliary cirrhosis classification|Classification]]==


In 2003 it was reported that an environmental Gram negative alphabacterium — ''[[Novosphingobium aromaticivorans]]''<ref name=Selmi2003>{{cite journal |author=Selmi C, Balkwill DL, Invernizzi P, ''et al.'' |title=Patients with primary biliary cirrhosis react against a ubiquitous xenobiotic-metabolizing bacterium |journal=Hepatology |volume=38 |issue=5 |pages=1250–7 |year=2003 |month=November |pmid=14578864 |doi=10.1053/jhep.2003.50446 }}</ref> was strongly associated with this disease. Subsequent reports appear to have confirmed this finding suggesting an aetiological role for this organism.<ref name=Mohammed2009>{{cite journal |author=Mohammed JP, Mattner J |title=Autoimmune disease triggered by infection with alphaproteobacteria |journal=Expert Rev Clin Immunol |volume=5 |issue=4 |pages=369–379 |year=2009 |month=July |pmid=20161124 |pmc=2742979 |doi=10.1586/ECI.09.23 }}</ref><ref name=Kaplan2004>{{cite journal |author=Kaplan MM |title=''Novosphingobium aromaticivorans'': a potential initiator of primary biliary cirrhosis |journal=Am. J. Gastroenterol. |volume=99 |issue=11 |pages=2147–9 |year=2004 |month=November |pmid=15554995 |doi=10.1111/j.1572-0241.2004.41121.x }}</ref><ref name=Selmi2004>{{cite journal |author=Selmi C, Gershwin ME |title=Bacteria and human autoimmunity: the case of primary biliary cirrhosis |journal=Curr Opin Rheumatol |volume=16 |issue=4 |pages=406–10 |year=2004 |month=July |pmid=15201604 |url=http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=1040-8711&volume=16&issue=4&spage=406}}</ref> The mechanism appears to be a cross reaction between the proteins of the bacterium and the mitochondrial proteins of the liver cells.<ref name=Mattner2008>{{cite journal |author=Mattner J, Savage PB, Leung P, ''et al.'' |title=Liver autoimmunity triggered by microbial activation of natural killer T cells |journal=Cell Host Microbe |volume=3 |issue=5 |pages=304–15 |year=2008 |month=May |pmid=18474357 |pmc=2453520 |doi=10.1016/j.chom.2008.03.009 |url=http://linkinghub.elsevier.com/retrieve/pii/S1931-3128(08)00120-0}}</ref> The gene encoding [[cluster of differentiation 101|CD101]] may also play a role in host susceptibility to this disease.<ref name=Mohammed2003>{{cite journal |author=Mohammed JP, Fusakio ME, Rainbow DB, ''et al.'' |title=Identification of Cd101 as a susceptibility gene for ''Novosphingobium aromaticivorans''-induced liver autoimmunity |journal=J. Immunol. |volume=187 |issue=1 |pages=337–49 |year=2011 |month=July |pmid=21613619 |pmc=3134939 |doi=10.4049/jimmunol.1003525 |url=http://www.jimmunol.org/cgi/pmidlookup?view=long&pmid=21613619}}</ref>
==[[Primary biliary cirrhosis pathophysiology|Pathophysiology]]==


==Epidemiology and Demographics==
==[[Primary biliary cirrhosis causes|Causes]]==
The female:male ratio is at least 9:1.  In some areas of the US and UK the prevalence is estimated to be as high as 1 in 4000. This is much more common than in South America or Africa, which may be due to better recognition in the US and UK.  First-degree relatives may have as much as a 500 times increase in prevalence, but there is debate if this risk is greater in the same generation relatives or the one that follows.
==Natural history, Complications and Prognosis==
===Complicatons===
Patients with primary biliary cirrhosis have an increased risk of [[hepatocellular carcinoma]].
===Prognosis===
The serum [[bilirubin]] level is an indicator of the prognosis of primary biliary cirrhosis, with levels of 2–6&nbsp;mg/dL having a mean [[survival time]] of 4.1 years, 6–10&nbsp;mg/dL having 2.1 years and those above 10&nbsp;mg/dL having a mean survival time of 1.4 years.<ref>[http://emedicine.medscape.com/article/171117-followup eMedicine > Primary Biliary Cirrhosis: Follow-up] Author: Nikolaos T Pyrsopoulos. Coauthor: K Rajender Reddy. Updated: Dec 23, 2009</ref>


'''[[Anti-nuclear antibodies]]''' appear to be prognostic agents in PBC. [[Anti-glycoprotein-210 antibodies]], and to a lessor degree [[anti-p62 antibodies]] correlate with progression toward end stage liver failure. [[Anti-centromere antibodies]] correlate with developing portal hypertension.<ref name="pmid17187436">{{cite journal | author = Nakamura M, Kondo H, Mori T, ''et al'' | title = Anti-gp210 and anti-centromere antibodies are different risk factors for the progression of primary biliary cirrhosis | journal = Hepatology | volume = 45 | issue = 1 | pages = 118-27 | year = 2007 | pmid = 17187436 | doi = 10.1002/hep.21472}}</ref>. Anti-np62<ref name="pmid11303304">{{cite journal | author = Nesher G, Margalit R, Ashkenazi YJ | title = Anti-nuclear envelope antibodies: Clinical associations | journal = Semin. Arthritis Rheum. | volume = 30 | issue = 5 | pages = 313-20 | year = 2001 | pmid = 11303304 | doi = 10.1053/sarh.2001.20266}}</ref> and anti-sp100 are also found in association with PBC.
==[[Primary biliary cirrhosis differential diagnosis|Differentiating Primary Biliary Cirrhosis from other Diseases]]==


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.<ref name="k&c-p429">Killenberg & Clavien (2006), p.&nbsp;429.</ref>
==[[Primary biliary cirrhosis epidemiology and demographics|Epidemiology and Demographics]]==
==Diagnosis==
===History and Symptoms===
The following symptoms may be present in PBC:
*[[Fatigue (physical)|Fatigue]]
*[[Pruritus]] (itchy skin)
*[[Jaundice]] (yellowing of the eyes and skin), due to increased [[bilirubin]] in the blood.
* Association with an extrahepatic autoimmune disorder such as Rheumatoid Arthritis or Sjögren syndrome (up to 80% incidence).
===Physical Examination===
On physical examination, the following may be present
*[[Xanthelasma]]ta (focal collections of [[cholesterol]] in the skin, especially around the eyes)
* Complications of [[cirrhosis]] and [[portal hypertension]]:
**[[Esophageal varices]]
** Fluid retention in the abdomen ([[ascites]])
** [[Hepatic encephalopathy]], up to coma, in extreme cases.


===Laboratory tests===
==[[Primary biliary cirrhosis risk factors|Risk Factors]]==
To diagnose PBC, distinctions should be established from other conditions with similar symptoms, such as [[autoimmune hepatitis]] or [[primary sclerosing cholangitis]] (PSC).


Diagnostic [[blood test]]s include:
==[[Primary biliary cirrhosis screening|Screening]]==
* Deranged [[liver function test]]s (high [[alkaline phosphatase]], elevated AST, ALT)
* Presence of certain [[antibodies]]: [[Anti-mitochondrial antibody|antimitochondrial antibody]] [[antinuclear antibody|antinuclear antibodies]] like [[Anti-glycoprotein-210 antibodies]], [[Anti-centromere antibodies]], anti-sp100.  (the M2-[[IgG]] antimitochondrial antibody is the most specific test)
===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.
==[[Primary biliary cirrhosis natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
====Summary of stages====
*''Stage 1 - Portal Stage'': Normal sized triads; portal inflammation, subtle [[bile duct]] damage.  [[Granuloma]]s are often detected in this stage.


*''Stage 2 - Periportal Stage'': Enlarged triads; periportal [[fibrosis]] and/or [[inflammation]]. Typically characterized by the finding of a proliferation of small bile ducts.
==Diagnosis==
 
*''Stage 3 - Septal Stage'': Active and/or passive fibrous [[septa]].
 
*''Stage 4 - Biliary Cirrhosis'': Nodules present; garland or jigsaw pattern.
===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'''
 
<gallery>
Image:


Primary biliary cirrhosis 001.jpg|T2
[[Primary biliary cirrhosis diagnostic study of choice|Diagnostic Study of Choice]] | [[Primary biliary cirrhosis history and symptoms|History and Symptoms]] | [[Primary biliary cirrhosis physical examination|Physical Examination]] | [[Primary biliary cirrhosis laboratory findings|Laboratory Findings]] |  [[Primary biliary cirrhosis electrocardiogram|Electrocardiogram]] |  [[Primary biliary cirrhosis x ray|X Ray]] | [[Primary biliary cirrhosis CT|CT]] | [[Primary biliary cirrhosis MRI|MRI]] | [[Primary biliary cirrhosis ultrasound|Ultrasound]] | [[Primary biliary cirrhosis other imaging findings|Other Imaging Findings]] | [[Primary biliary cirrhosis other diagnostic studies|Other Diagnostic Studies]]


Image:
==Treatment==
Primary biliary cirrhosis 002.jpg|T2Image:
Primary biliary cirrhosis 003.jpg|T1 preImage:
Primary biliary cirrhosis 004.jpg|T1 postImage:
Primary biliary cirrhosis 005.jpg|T1 postImage:
Primary biliary cirrhosis 006.jpg|T1 postImage:
Primary biliary cirrhosis 007.jpg|T1 post</gallery>


==Therapy==
[[Primary biliary cirrhosis medical therapy|Medical Therapy]] | [[Primary biliary cirrhosis surgery|Surgery]] | [[Primary biliary cirrhosis primary prevention|Primary Prevention]] | [[Primary biliary cirrhosis secondary prevention|Secondary Prevention]] | [[Primary biliary cirrhosis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Primary biliary cirrhosis future or investigational therapies|Future or Investigational Therapies]]
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.
==Case Studies==


* 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]].
[[Primary biliary cirrhosis case study one|Case #1]]
 
==Related Chapters==
* To relieve fatigue associated with primary biliary cirrhosis, current studies indicate that Provigil (modafinil) may be effective without damaging the liver.<ref>[[Modafinil#Primary_biliary_cirrhosis]]<br/>{{cite journal |author=Ian Gan S, de Jongh M, Kaplan MM |title=Modafinil in the treatment of debilitating fatigue in primary biliary cirrhosis: a clinical experience |journal=Dig. Dis. Sci. |volume=54 |issue=10 |pages=2242–6 |year=2009 |month=October |pmid=19082890 |doi=10.1007/s10620-008-0613-3 |url=http://www.springerlink.com/content/f0207x6110847113/}}<br/>{{cite journal |author=Kumagi T, Heathcote EJ |title=Primary biliary cirrhosis |journal=Orphanet J Rare Dis |volume=3 |pages=1 |year=2008 |pmid=18215315 |pmc=2266722 |doi=10.1186/1750-1172-3-1 |url=http://www.ojrd.com/content/3//1 |quote=[http://www.ojrd.com/content/3/1/1#B157 Ref 157 viz:]}}<br/>{{cite journal |author=Jones DE, Newton JL |title=An open study of modafinil for the treatment of daytime somnolence and fatigue in primary biliary cirrhosis |journal=Aliment. Pharmacol. Ther. |volume=25 |issue=4 |pages=471–6 |year=2007 |month=February |pmid=17270003 |doi=10.1111/j.1365-2036.2006.03223.x |url=http://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0269-2813&date=2007&volume=25&issue=4&spage=471}}</ref>  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.<ref>[[Modafinil#Patent_protection_and_antitrust_litigation]]<br/>{{cite journal |author=Carrier MA |title=Provigil: A Case Study of Anticompetitive Behavior |journal=Hastings Science & Technology Law Journal |volume=3 |issue=2 |pages=441–452 |year=2011 |url=http://hstlj.org/content/vol3/iss2/v3i2carrier.pdf |format=PDF}}</ref>  The FTC has filed suit against Cephalon alleging anti-competitive behavior.<ref>http://www.ftc.gov/os/caselist/0610182/080213complaint.pdf</ref>
 
* Patients with PBC have poor lipid-dependent absorption of Vitamins A, D, E, K.<ref name="BaconO'Grady2006">{{cite book|author1=Bruce R. Bacon|author2=John G. O'Grady|title=Comprehensive clinical hepatology|url=http://books.google.com/?id=ec0G9HGiR8MC&pg=PA283|accessdate=29 June 2010|year=2006|publisher=Elsevier Health Sciences|isbn=978-0-323-03675-7|pages=283–}}</ref> Appropriate supplementation is recommended when bilirubin is elevated<ref>{{cite journal|last=Lindor|first=KD|coauthors=Gershwin, ME; Poupon, R; Kaplan, M; Bergasa, NV; Heathcote, EJ; American Association for Study of Liver, Diseases|title=Primary biliary cirrhosis.|journal=Hepatology (Baltimore, Md.)|date=2009 Jul|volume=50|issue=1|pages=291-308|pmid=19554543|doi=10.1002/hep.22906}}</ref>. Multivitamins (esp. Vitamin D) and calcium are also recommended.
 
* Patients with PBC are at elevated risk of developing [[osteoporosis]]<ref>{{cite journal|last=Collier|first=Jane|title=Guidelines on the management of osteoporosis associated with chronic liver disease|journal=Gut|year=2002|volume=50|pages=i1-i9|pmid=11788576|accessdate=14 June 2012|pmc=1867644}}</ref> and [[esophageal varices]]<ref>{{cite journal|last=Ali|first=AH|coauthors=Sinakos, E; Silveira, MG; Jorgensen, RA; Angulo, P; Lindor, KD|title=Varices in early histological stage primary biliary cirrhosis.|journal=Journal of Clinical Gastroenterology|date=2011 Aug|volume=45|issue=7|pages=e66-71|pmid=20856137|doi=10.1097/MCG.0b013e3181f18c4e}}</ref>  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, [[alcoholic beverage]]s are contraindicated.
 
As in all liver diseases, excessive consumption of [[alcohol]] is contraindicated.
 
In advanced cases, a [[Liver transplantation|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 <ref>Medical care of the Liver Trasplant Patient, 3rd Edition published 2006, editied by Paul G. Killenberg, page 429</ref>
 
[[Obeticholic acid]] is in phase III clinical trials for PBC.<ref>http://www.genengnews.com/gen-news-highlights/dainippon-sumitomo-pays-intercept-15m-for-phase-iii-liver-disease-drug/81244901/</ref>
 
==References==
<div class="references-small"><references/></div>
 
==Sources==
===Medical===
*{{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.  [http://www.liverdisease.com her webpage].
*Howard J. Worman. ''The Liver Disorders Sourcebook'',  McGraw-Hill, 1999, ISBN 0-7373-0090-6.
 
==See also==
*[[Bile canaliculus]]
*[[Bile canaliculus]]
<br>
[[de:Primär biliäre Zirrhose]]
[[pl:Pierwotna marskość żółciowa wątroby]]
[[sv:primär biliär cirrhos]]


==External links==
* [http://pbcers.org PBCers.org] - patients' organisation
* [http://www.pbcfoundation.org.uk/ PBC Foundation UK]


{{WikiDoc Help Menu}}
{{WikiDoc Sources}}


[[Category:Gastroenterology]]
[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]
[[Category:Disease]]
[[de:Primär biliäre Zirrhose]]
[[Category:Rheumatology]]
[[pl:Pierwotna marskość żółciowa wątroby]]
[[Category:Medicine]]
[[sv:primär biliär cirrhos]]
[[Category:Up-To-Date]]
[[Category:Grammar]]
 
{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

Latest revision as of 23:49, 29 July 2020


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: Anmol Pitliya, M.B.B.S. M.D.[2], Dildar Hussain, MBBS [3], Aysha Anwar, M.B.B.S[4], Aravind Reddy Kothagadi M.B.B.S[5]

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

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

Related Chapters


de:Primär biliäre Zirrhose sv:primär biliär cirrhos


Template:WikiDoc Sources