wikidoc - User contributions [en]

Primary biliary cirrhosis

2013-04-16T20:23:50Z

Aditya Govindavarjhulla: /* Related Chapters */

Primary biliary cirrhosis other diagnostic studies

2013-04-16T20:21:40Z

Aditya Govindavarjhulla: /* Liver Biopsy */

__NOTOC__
{{CMG}}
{{Primary biliary cirrhosis}}

==Other Diagnostic Studies==
===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
*Periductal [[epithelioid]] [[granulomata]]

A liver biopsy is necessary to determine the stage of disease.

===ERCP===
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]]).

==References==
{{reflist|2}}

{{WH}}
{{WS}}

[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]

[[Category:Needs overview]]

Primary biliary cirrhosis medical therapy

2013-04-16T20:21:02Z

Aditya Govindavarjhulla: /* Medical Therapy */

__NOTOC__
{{CMG}}
{{Primary biliary cirrhosis}}
==Medical 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.<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.

==References==
{{reflist|2}}

{{WH}}
{{WS}}

[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]

[[Category:Needs overview]]

Primary biliary cirrhosis CT

2013-04-16T20:20:04Z

Aditya Govindavarjhulla:

__NOTOC__
{{CMG}}
{{Primary biliary cirrhosis}}
Please help WikiDoc by adding more content here. It's easy! Click [[help:How to Edit a Page|here]] to learn about editing.
==Overview==
[[CT scan]] or abdominal [[medical ultrasonography|ultrasound]] is usually performed to rule out blockage to the bile ducts.
==References==
{{reflist|2}}

{{WH}}
{{WS}}

[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]

[[Category:Needs content]]

Primary biliary cirrhosis overview

2013-04-16T20:19:17Z

Aditya Govindavarjhulla: /* Epidemiology and Demographics */

__NOTOC__
{{Primary biliary cirrhosis}}
{{CMG}}

==Overview==
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>
==Epidemiology and Demographics==
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.
==Diagnosis==
===CT===
Abdominal [[medical ultrasonography|ultrasound]] or a [[CT scan]] is usually performed to rule out blockage to the bile ducts.

==Treatment==
===Surgery===
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>
===Future or Investigational Therapies===
[[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==
{{reflist|2}}
{{WH}}
{{WS}}

[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]

Primary biliary cirrhosis CT

2013-04-16T20:17:47Z

Aditya Govindavarjhulla: Created page with "__NOTOC__ {{Primary biliary cirrhosis}} Please help WikiDoc by adding content here. It's easy! Click here to learn about editing. ==References== {..."

__NOTOC__
{{Primary biliary cirrhosis}}
Please help WikiDoc by adding content here. It's easy! Click [[help:How to Edit a Page|here]] to learn about editing.
==References==
{{reflist|2}}

{{WH}}
{{WS}}

[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]

[[Category:Needs content]]

Primary biliary cirrhosis

2013-04-16T20:14:44Z

Aditya Govindavarjhulla: /* Related Chapters */

Primary biliary cirrhosis

2013-04-16T20:14:30Z

Aditya Govindavarjhulla: /* Treatment */

Primary biliary cirrhosis

2013-04-16T20:14:07Z

Aditya Govindavarjhulla: /* Diagnosis */

Primary biliary cirrhosis

2013-04-16T20:13:35Z

Aditya Govindavarjhulla:

Primary biliary cirrhosis

2013-04-16T20:12:59Z

Aditya Govindavarjhulla:

Primary biliary cirrhosis

2013-04-16T20:11:09Z

Aditya Govindavarjhulla: /* External Links */

Primary biliary cirrhosis

2013-04-16T20:10:36Z

Aditya Govindavarjhulla:

Template:Primary biliary cirrhosis

2013-04-16T20:09:29Z

Aditya Govindavarjhulla:

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'''Primary Biliary Cirrhosis Microchapters
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[[Primary biliary cirrhosis|Home]]
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[[Primary biliary cirrhosis (patient information)|Patient Information]]
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[[Primary biliary cirrhosis overview|Overview]]
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[[Primary biliary cirrhosis historical perspective|Historical Perspective]]
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[[Primary biliary cirrhosis classification|Classification]]
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[[Primary biliary cirrhosis pathophysiology|Pathophysiology]]
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[[Primary biliary cirrhosis causes|Causes]]
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[[Primary biliary cirrhosis differential diagnosis|Differentiating Primary Biliary Cirrhosis from other Diseases]]
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[[Primary biliary cirrhosis epidemiology and demographics|Epidemiology and Demographics]]
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[[Primary biliary cirrhosis risk factors|Risk Factors]]
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[[Primary biliary cirrhosis screening|Screening]]
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[[Primary biliary cirrhosis natural history, complications and prognosis|Natural History, Complications and Prognosis]]
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Diagnosis
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[[Primary biliary cirrhosis history and symptoms|History and Symptoms]]
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[[Primary biliary cirrhosis physical examination|Physical Examination]]
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[[Primary biliary cirrhosis laboratory findings|Laboratory Findings]]
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[[Primary biliary cirrhosis CT|CT]]
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[[Primary biliary cirrhosis MRI|MRI]]
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[[Primary biliary cirrhosis ultrasound|Ultrasound]]
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[[Primary biliary cirrhosis other imaging findings|Other Imaging Findings]]
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[[Primary biliary cirrhosis other diagnostic studies|Other Diagnostic Studies]]
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Treatment
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[[Primary biliary cirrhosis medical therapy|Medical Therapy]]
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[[Primary biliary cirrhosis surgery|Surgery]]
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[[Primary biliary cirrhosis primary prevention|Primary Prevention]]
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[[Primary biliary cirrhosis secondary prevention|Secondary Prevention]]
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[[Primary biliary cirrhosis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]]
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[[Primary biliary cirrhosis future or investigational therapies|Future or Investigational Therapies]]
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Case Studies
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[[Primary biliary cirrhosis case study one|Case #1]]
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{{PAGENAME}} On the Web
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[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?CMD=search&db=pubmed&term={{urlencode:{{#if:{{{1|}}}|{{{1}}}|{{PAGENAME}}}}}} Most recent articles]
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Primary biliary cirrhosis cost-effectiveness of therapy

2013-04-16T20:08:33Z

Aditya Govindavarjhulla: Created page with "__NOTOC__ {{Primary biliary cirrhosis}} Please help WikiDoc by adding content here. It's easy! Click here to learn about editing. ==References== {..."

Primary biliary cirrhosis secondary prevention

2013-04-16T20:08:21Z

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Primary biliary cirrhosis primary prevention

2013-04-16T20:08:09Z

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Primary biliary cirrhosis other imaging findings

2013-04-16T20:07:51Z

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Primary biliary cirrhosis ultrasound

2013-04-16T20:07:12Z

Aditya Govindavarjhulla: Aditya Govindavarjhulla moved page Primary biliary cirrhosis echocardiography or ultrasound to Primary biliary cirrhosis ultrasound

Primary biliary cirrhosis ultrasound

2013-04-16T20:06:57Z

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Primary biliary cirrhosis screening

2013-04-16T20:06:29Z

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Primary biliary cirrhosis risk factors

2013-04-16T20:06:16Z

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Primary biliary cirrhosis differential diagnosis

2013-04-16T20:06:05Z

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Primary biliary cirrhosis causes

2013-04-16T20:05:55Z

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Primary biliary cirrhosis classification

2013-04-16T20:05:45Z

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Primary biliary cirrhosis

2013-04-16T20:04:56Z

Aditya Govindavarjhulla:

Primary biliary cirrhosis

2013-04-16T20:04:30Z

Aditya Govindavarjhulla: /* Sources */

Primary biliary cirrhosis overview

2013-04-16T20:04:06Z

Aditya Govindavarjhulla: /* Surgery */

__NOTOC__
{{Primary biliary cirrhosis}}
{{CMG}}

==Overview==
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>
==Epidemiology and Demographics==
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.

==Treatment==
===Surgery===
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>
===Future or Investigational Therapies===
[[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==
{{reflist|2}}
{{WH}}
{{WS}}

[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]

Primary biliary cirrhosis future or investigational therapies

2013-04-16T20:03:44Z

Aditya Govindavarjhulla:

__NOTOC__
{{CMG}}
{{Primary biliary cirrhosis}}
Please help WikiDoc by adding more content here. It's easy! Click [[help:How to Edit a Page|here]] to learn about editing.
==Overview==
[[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==
{{reflist|2}}

{{WH}}
{{WS}}

[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]

[[Category:Needs content]]

Primary biliary cirrhosis

2013-04-16T20:03:32Z

Aditya Govindavarjhulla: /* Therapy */

Primary biliary cirrhosis future or investigational therapies

2013-04-16T20:03:18Z

Aditya Govindavarjhulla: Created page with "__NOTOC__ {{CMG}} {{Primary biliary cirrhosis}} Please help WikiDoc by adding more content here. It's easy! Click here to learn about editing. ==O..."

__NOTOC__
{{CMG}}
{{Primary biliary cirrhosis}}
Please help WikiDoc by adding more content here. It's easy! Click [[help:How to Edit a Page|here]] to learn about editing.
==Overview==

==References==
{{reflist|2}}

{{WH}}
{{WS}}

[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]

[[Category:Needs content]]

Primary biliary cirrhosis overview

2013-04-16T20:02:43Z

Aditya Govindavarjhulla: /* References */

__NOTOC__
{{Primary biliary cirrhosis}}
{{CMG}}

==Overview==
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>
==Epidemiology and Demographics==
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.

==Treatment==
===Surgery===
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>
==References==
{{reflist|2}}
{{WH}}
{{WS}}

[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]

Primary biliary cirrhosis surgery

2013-04-16T20:02:13Z

Aditya Govindavarjhulla:

__NOTOC__
{{CMG}}
{{Primary biliary cirrhosis}}
Please help WikiDoc by adding more content here. It's easy! Click [[help:How to Edit a Page|here]] to learn about editing.
==Overview==
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>
==References==
{{reflist|2}}

{{WH}}
{{WS}}

[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]
[[Category:Needs content]]

Primary biliary cirrhosis

2013-04-16T20:01:58Z

Aditya Govindavarjhulla: /* Therapy */

Primary biliary cirrhosis surgery

2013-04-16T20:01:41Z

Aditya Govindavarjhulla: Created page with "__NOTOC__ {{CMG}} {{Primary biliary cirrhosis}} Please help WikiDoc by adding more content here. It's easy! Click here to learn about editing. ==O..."

__NOTOC__
{{CMG}}
{{Primary biliary cirrhosis}}
Please help WikiDoc by adding more content here. It's easy! Click [[help:How to Edit a Page|here]] to learn about editing.
==Overview==

==References==
{{reflist|2}}

{{WH}}
{{WS}}

[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]
[[Category:Needs content]]

Primary biliary cirrhosis medical therapy

2013-04-16T20:00:47Z

Aditya Govindavarjhulla: /* Medical Therapy */

__NOTOC__
{{CMG}}
{{Primary biliary cirrhosis}}
==Medical 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.<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.

==References==
{{reflist|2}}

{{WH}}
{{WS}}

[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]

[[Category:Needs overview]]

Primary biliary cirrhosis medical therapy

2013-04-16T20:00:03Z

Aditya Govindavarjhulla:

__NOTOC__
{{CMG}}
{{Primary biliary cirrhosis}}
==Medical 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.<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.
==References==
{{reflist|2}}

{{WH}}
{{WS}}

[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]

[[Category:Needs overview]]

Primary biliary cirrhosis

2013-04-16T19:59:47Z

Aditya Govindavarjhulla: /* Therapy */

Primary biliary cirrhosis medical therapy

2013-04-16T19:58:59Z

Aditya Govindavarjhulla: Created page with "__NOTOC__ {{CMG}} {{Primary biliary cirrhosis}} Please help WikiDoc by adding content here. It's easy! Click here to learn about editing. ==Medical..."

__NOTOC__
{{CMG}}
{{Primary biliary cirrhosis}}
Please help WikiDoc by adding content here. It's easy! Click [[help:How to Edit a Page|here]] to learn about editing.
==Medical Therapy==

==References==
{{reflist|2}}

{{WH}}
{{WS}}

[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]

[[Category:Needs overview]]

Primary biliary cirrhosis MRI

2013-04-16T19:57:38Z

Aditya Govindavarjhulla: /* MRI= */

__NOTOC__
{{CMG}}
{{Primary biliary cirrhosis}}
Please help WikiDoc by adding more content here. It's easy! Click [[help:How to Edit a Page|here]] to learn about editing.
==MRI==
'''MR images of a patient with primary biliary cirrhosis'''

<gallery>
Image:

Primary biliary cirrhosis 001.jpg|T2

Image:
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>

==References==
{{reflist|2}}

{{WH}}
{{WS}}

[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]
[[Category:needs overview]]
[[Category:Needs content]]

Primary biliary cirrhosis MRI

2013-04-16T19:57:16Z

Aditya Govindavarjhulla:

__NOTOC__
{{CMG}}
{{Primary biliary cirrhosis}}
Please help WikiDoc by adding more content here. It's easy! Click [[help:How to Edit a Page|here]] to learn about editing.
==MRI===
'''MR images of a patient with primary biliary cirrhosis'''

<gallery>
Image:

Primary biliary cirrhosis 001.jpg|T2

Image:
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>
==References==
{{reflist|2}}

{{WH}}
{{WS}}

[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]
[[Category:needs overview]]
[[Category:Needs content]]

Primary biliary cirrhosis

2013-04-16T19:56:47Z

Aditya Govindavarjhulla:

__NOTOC__
'''For patient information page on this topic, click [[Primary biliary cirrhosis (patient information)|here]].'''
{{Primary biliary cirrhosis}}

{{CMG}} '''Associate Editor(s)-In-Chief:''' {{CZ}} [[User: Prashanthsaddala|Prashanth Saddala M.B.B.S]]

{{SK}} Chronic non-suppurative destructive cholangitis, PBC
==Causes==

==Diagnosis==

==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.<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]]

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

[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]

[[de:Primär biliäre Zirrhose]]
[[pl:Pierwotna marskość żółciowa wątroby]]
[[sv:primär biliär cirrhos]]
[[Category:Grammar]]

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Primary biliary cirrhosis MRI

2013-04-16T19:55:58Z

Aditya Govindavarjhulla: Created page with "__NOTOC__ {{CMG}} {{Primary biliary cirrhosis}} Please help WikiDoc by adding more content here. It's easy! Click here to learn about editing. ==R..."

__NOTOC__
{{CMG}}
{{Primary biliary cirrhosis}}
Please help WikiDoc by adding more content here. It's easy! Click [[help:How to Edit a Page|here]] to learn about editing.
==References==
{{reflist|2}}

{{WH}}
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[[Category:Gastroenterology]]
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Primary biliary cirrhosis other diagnostic studies

2013-04-16T19:55:07Z

Aditya Govindavarjhulla:

__NOTOC__
{{CMG}}
{{Primary biliary cirrhosis}}

==Other Diagnostic Studies==
===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.
===ERCP===
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]]).

==References==
{{reflist|2}}

{{WH}}
{{WS}}

[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]

[[Category:Needs overview]]

Primary biliary cirrhosis

2013-04-16T19:54:15Z

Aditya Govindavarjhulla: /* Diagnosis */

__NOTOC__
'''For patient information page on this topic, click [[Primary biliary cirrhosis (patient information)|here]].'''
{{Primary biliary cirrhosis}}

{{CMG}} '''Associate Editor(s)-In-Chief:''' {{CZ}} [[User: Prashanthsaddala|Prashanth Saddala M.B.B.S]]

{{SK}} Chronic non-suppurative destructive cholangitis, PBC
==Causes==

==Diagnosis==

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

<gallery>
Image:

Primary biliary cirrhosis 001.jpg|T2

Image:
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==
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.<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]]

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

[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]

[[de:Primär biliäre Zirrhose]]
[[pl:Pierwotna marskość żółciowa wątroby]]
[[sv:primär biliär cirrhos]]
[[Category:Grammar]]

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Primary biliary cirrhosis other diagnostic studies

2013-04-16T19:53:31Z

Aditya Govindavarjhulla: Created page with "__NOTOC__ {{CMG}} {{Primary biliary cirrhosis}} Please help WikiDoc by adding content here. It's easy! Click here to learn about editing. ==Other D..."

__NOTOC__
{{CMG}}
{{Primary biliary cirrhosis}}
Please help WikiDoc by adding content here. It's easy! Click [[help:How to Edit a Page|here]] to learn about editing.
==Other Diagnostic Studies==

==References==
{{reflist|2}}

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Primary biliary cirrhosis laboratory findings

2013-04-16T19:52:06Z

Aditya Govindavarjhulla: /* Laboratory Findings */

__NOTOC__
{{CMG}}
{{Primary biliary cirrhosis}}

==Laboratory Findings==
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:
* 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)

==References==
{{reflist|2}}

{{WH}}
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[[Category:Gastroenterology]]
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[[Category:Disease]]

[[Category:Needs overview]]

Primary biliary cirrhosis laboratory findings

2013-04-16T19:51:19Z

Aditya Govindavarjhulla:

__NOTOC__
{{CMG}}
{{Primary biliary cirrhosis}}

===Laboratory Findings===
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:
* 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)

==References==
{{reflist|2}}

{{WH}}
{{WS}}

[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]

[[Category:Needs overview]]

Primary biliary cirrhosis

2013-04-16T19:50:22Z

Aditya Govindavarjhulla: /* Laboratory tests */

__NOTOC__
'''For patient information page on this topic, click [[Primary biliary cirrhosis (patient information)|here]].'''
{{Primary biliary cirrhosis}}

{{CMG}} '''Associate Editor(s)-In-Chief:''' {{CZ}} [[User: Prashanthsaddala|Prashanth Saddala M.B.B.S]]

{{SK}} Chronic non-suppurative destructive cholangitis, PBC
==Causes==

==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'''

<gallery>
Image:

Primary biliary cirrhosis 001.jpg|T2

Image:
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==
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.<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]]

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

[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]

[[de:Primär biliäre Zirrhose]]
[[pl:Pierwotna marskość żółciowa wątroby]]
[[sv:primär biliär cirrhos]]
[[Category:Grammar]]

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

Primary biliary cirrhosis laboratory findings

2013-04-16T19:50:08Z

Aditya Govindavarjhulla: Created page with "__NOTOC__ {{CMG}} {{Primary biliary cirrhosis}} Please help WikiDoc by adding content here. It's easy! Click here to learn about editing. ==Refere..."

__NOTOC__
{{CMG}}
{{Primary biliary cirrhosis}}
Please help WikiDoc by adding content here. It's easy! Click [[help:How to Edit a Page|here]] to learn about editing.
==References==
{{reflist|2}}

{{WH}}
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[[Category:Gastroenterology]]
[[Category:Hepatology]]
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