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__NOTOC__
{{DiseaseDisorder infobox |
{{DiseaseDisorder infobox |
   Name        = Primary sclerosing cholangitis |
   Name        = Primary sclerosing cholangitis |
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'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''


{{CMG}}
{{CMG}}; {{AE}}{{DN}}
==Overview==


'''Primary sclerosing cholangitis''' ('''PSC''') is a form of [[cholangitis]] due to an [[autoimmune]] reaction. A cholangitis is an [[inflammation]] of the [[bile duct]]s of the [[liver]]. Primary sclerosing cholangitis leads to [[cholestasis]] (blockage of [[bile]] transport to the gut). Blockage of the bile duct leads to accumulation of bile, which damages the liver, leading to [[jaundice]] and eventually causes [[liver failure]].
{{SK}} PSC; primary sclerosing cholangitis
==[[Primary sclerosing cholangitis overview|Overview]]==


==Signs and symptoms==
==[[Primary sclerosing cholangitis historical perspective|Historical Perspective]]==
* Tiredness (a non-specific symptom often present in liver disease)
* Severe [[jaundice]] with intense [[itch]]ing
* [[Malabsorption]] (especially of [[fat]]) and [[steatorrhea]], leading to decreased levels of the fat-soluble [[vitamin]]s, [[vitamin A|A]], [[vitamin D|D]], [[vitamin E|E]] and [[vitamin K|K]].
* Signs of [[cirrhosis]]
* [[Ascending cholangitis]], or [[infection]] of the bile duct.


==Diagnosis==
==[[Primary sclerosing cholangitis classification|Classification]]==
The diagnosis is by imaging of the bile duct, usually in the setting of [[endoscopic retrograde cholangiopancreatography]] (ERCP, [[endoscopy]] of the bile duct and [[pancreas]]), which shows characteristic changes ("beading") of the bile ducts. Another option is [[magnetic resonance cholangiopancreatography]] (MRCP), where [[magnetic resonance imaging]] is used to visualise the biliary tract.


Other tests often done are a [[full blood count]], [[liver enzyme]]s, [[bilirubin]] levels (usually grossly elevated), [[renal function]], [[electrolyte]]s. [[Fecal fat]] determination is occasionally ordered when the symptoms of malabsorption are prominent.
==[[Primary sclerosing cholangitis pathophysiology|Pathophysiology]]==


The differential diagnosis can include [[primary biliary cirrhosis]], drug induced [[cholestasis]], [[cholangiocarcinoma]], and [[HIV]]-associated cholangiopathy.
==[[Primary sclerosing cholangitis causes|Causes]]==


==Etiology==
==[[Primary sclerosing cholangitis differential diagnosis|Differentiating Primary sclerosing cholangitis from other Diseases]]==
The cause(s) for PSC are unknown. It is often considered to be an [[autoimmunity|autoimmune disorder]]. PSC is associated with [[ulcerative colitis]]. It is assumed that these diseases share a common cause.


Ulcerative colitis is a systemic disease that affects many areas of the body. PSC is often listed as a manifestation of ulcerative colitis outside the colon. PSC differs from these manifestations in that, unlike most other manifestations, PSC continues in spite of surgical removal of the colon.[http://www.acg.gi.org] This suggests that, while the cause of ulcerative colitis, and most of its manifestations, is in the colon, the cause of PSC is located outside the colon.
==[[Primary sclerosing cholangitis epidemiology and demographics|Epidemiology and Demographics]]==


==Pathophysiology==
==[[Primary sclerosing cholangitis risk factors|Risk Factors]]==
Bile ducts, both intra- and extrahepatically (inside the liver and outside), are inflamed and develop scarring, obstructing the flow of bile. As bile assists in the enteric breakdown and absorption of fat, the absence of bile leads to fat malabsorption. The bile accumulates in the duct, leading to liver cell damage and liver failure.


==Epidemiology==
==[[Primary sclerosing cholangitis screening|Screening]]==
It is more prevalent in men than in women. The disease normally starts from age 30 to 60. It can however also start with children. PSC progresses slowly, so the [[disease]] can be active for a long time before it is noticed or diagnosed.


==Screening==
==[[Primary sclerosing cholangitis natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
PSC is associated with [[cholangiocarcinoma]], which are tumors involving the biliary tree.  Screening for cholangiocarcinoma in patients with PSC is encouraged, but there is no general consensus on the modality and interval of choice.


==Therapy==
==[[Primary sclerosing cholangitis diagnosis|Diagnosis]]==
Standard treatment includes [[ursodiol]], a bile acid naturally produced by the liver, which has been shown to lower elevated liver enzyme numbers in people with PSC, but has not yet been proven effective at prolonging the life of the liver.  Treatment also includes medication to relieve itching ([[antipruritic]]s) and bile acid sequesterants (cholestyramine), [[antibiotic]]s to treat infections, and [[vitamin]] supplements, as people with PSC are often deficient in [[vitamin A]], [[vitamin D]], and [[vitamin K]].
[[Primary sclerosing cholangitis history and symptoms|History and Symptoms]] | [[Primary sclerosing cholangitis physical examination|Physical Examination]] | [[Primary sclerosing cholangitis laboratory findings|Laboratory Findings]] | [[Primary sclerosing cholangitis electrocardiogram|Electrocardiogram]] | [[Primary sclerosing cholangitis x ray|X Ray]] | [[Primary sclerosing cholangitis CT|CT]] | [[Primary sclerosing cholangitis MRI|MRI]] | [[Primary sclerosing cholangitis ultrasound|Ultrasound]] | [[Primary sclerosing cholangitis other imaging findings|Other Imaging Findings]] | [[Primary sclerosing cholangitis other diagnostic studies|Other Diagnostic Studies]]


In some cases, ERCP, which may involve stenting of the common bile duct, may be necessary in order to open major blockages (dominant strictures).
==Treatment==
[[Primary sclerosing cholangitis medical therapy|Medical Therapy]] | [[Primary sclerosing cholangitis surgery|Surgery]] | [[Primary sclerosing cholangitis primary prevention|Primary Prevention]] | [[Primary sclerosing cholangitis secondary prevention|Secondary Prevention]] | [[Primary sclerosing cholangitis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Primary sclerosing cholangitis future or investigational therapies|Future or Investigational Therapies]]


[[Liver transplantation]] (including live transplants whereby a portion of a living donor is given to the recipient) is an option if the liver begins to fail.
==Case Studies==
 
[[Primary sclerosing cholangitis case study one|Case #1]]


== See also ==
== See also ==
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* [[Alagille syndrome]]
* [[Alagille syndrome]]
* [[Progressive familial intrahepatic cholestasis]]
* [[Progressive familial intrahepatic cholestasis]]
* Walter Payton (died of primary sclerosing cholangitis)


== External links ==
== External links ==
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{{Gastroenterology}}
{{Gastroenterology}}


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


[[de:Cholangitis]]
[[de:Cholangitis]]

Latest revision as of 18:16, 12 December 2016

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Dima Nimri, M.D. [2]

Synonyms and keywords: PSC; primary sclerosing cholangitis

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Primary sclerosing cholangitis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

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

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Case #1

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