Primary sclerosing cholangitis

Common Name(s)

Primary sclerosing cholangitis

Primary Sclerosing Cholangitis (PSC) is a rare liver and bile duct disease which may have an autoimmune component. It's not curable, but some of the symptoms are treatable - and there's hope. PSC is often associated with ulcerative colitis, Crohn’s disease, and related gastrointestinal and autoimmune diseases. Some PSC patients eventually require a liver transplant. Many PSCers, despite daily limitations and medications, are able to live full lives.

Source: Advocacy organizations associated with the condition.

 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Primary sclerosing cholangitis" for support, advocacy or research.

PSC Partners Seeking a Cure

PSC Partners Seeking a Cure is a 501(c)3 nonprofit foundation whose mission is to provide education and support to PSC patients and their families, raise funds with which to research PSC, find treatments and promote awareness of PSC. The organization holds an annual three-day conference at various PSC centers in the US. Attendees come from all over the world. PSC Partners provides online support to patients, and has an important PSC and related diseases literature site. PSC Partners has a competitive research grant program and quarterly newsletters. Our website is a hub for PSCers.

Last Updated: 4 Dec 2012

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General Support Organizations

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Primary sclerosing cholangitis" for support, advocacy or research.

PSC Partners Seeking a Cure

PSC Partners Seeking a Cure is a 501(c)3 nonprofit foundation whose mission is to provide education and support to PSC patients and their families, raise funds with which to research PSC, find treatments and promote awareness of PSC. The organization holds an annual three-day conference at various PSC centers in the US. Attendees come from all over the world. PSC Partners provides online support to patients, and has an important PSC and related diseases literature site. PSC Partners has a competitive research grant program and quarterly newsletters. Our website is a hub for PSCers.

http://www.pscpartners.org

Last Updated: 4 Dec 2012

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Primary sclerosing cholangitis" returned 209 free, full-text research articles on human participants. First 3 results:

Characterization of cultured cholangiocytes isolated from livers of patients with primary sclerosing cholangitis.
 

Author(s): James H Tabibian, Christy E Trussoni, Steven P O'Hara, Patrick L Splinter, Julie K Heimbach, Nicholas F LaRusso

Journal: Lab. Invest.. 2014 Oct;94(10):1126-33.

 

Primary sclerosing cholangitis (PSC) is a chronic, idiopathic cholangiopathy. The role of cholangiocytes (biliary epithelial cells) in PSC pathogenesis is unknown and remains an active area of research. Here, through cellular, molecular and next-generation sequencing (NGS) methods, ...

Last Updated: 2 Oct 2014

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Association between serum IgE level and adverse clinical endpoints in primary sclerosing cholangitis.
 

Author(s): James H Tabibian, Felicity Enders, Mohamad H Imam, Gururaj Kolar, Keith D Lindor, Jayant A Talwalkar

Journal: Ann Hepatol. ;13(3):384-9.

 

Primary sclerosing cholangitis (PSC) is an idiopathic hepatobiliary disorder associated with an increased risk for cholangiocarcinoma (CCA) and a median survival time of 12 years. Reliable predictors of CCA and other major adverse events in PSC are currently lacking. Recently, serum ...

Last Updated: 23 Apr 2014

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Primary sclerosing cholangitis in patient with celiac disease complicated by cholecystic empyema and acute pancreatitis.
 

Author(s): P Piccolboni, E Ragone, A Inzirillo, R Utili

Journal: G Chir. ;34(9-10):267-70.

 

The association of celiac disease and sclerosing cholangitis is a well known, although unusual, pathologic feature of autoimmunity.

Last Updated: 17 Mar 2014

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Primary sclerosing cholangitis" returned 44 free, full-text review articles on human participants. First 3 results:

Characterization of animal models for primary sclerosing cholangitis (PSC).
 

Author(s): Peter Fickert, Marion J Pollheimer, Ulrich Beuers, Carolin Lackner, Gideon Hirschfield, Chantal Housset, Verena Keitel, Christoph Schramm, Hanns-Ulrich Marschall, Tom H Karlsen, Espen Melum, Arthur Kaser, Bertus Eksteen, Mario Strazzabosco, Michael Manns, Michael Trauner,

Journal: J. Hepatol.. 2014 Jun;60(6):1290-303.

 

Primary sclerosing cholangitis (PSC) is a chronic cholangiopathy characterized by biliary fibrosis, development of cholestasis and end stage liver disease, high risk of malignancy, and frequent need for liver transplantation. The poor understanding of its pathogenesis is also reflected ...

Last Updated: 19 May 2014

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Role of the microbiota and antibiotics in primary sclerosing cholangitis.
 

Author(s): James H Tabibian, Jayant A Talwalkar, Keith D Lindor

Journal: Biomed Res Int. 2013 ;2013():389537.

 

Primary sclerosing cholangitis (PSC) is an idiopathic, progressive, cholestatic liver disease with considerable morbidity and mortality and no established pharmacotherapy. In addition to the long-recognized association between PSC and inflammatory bowel disease, several lines of preclinical ...

Last Updated: 15 Nov 2013

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Pathogenesis of primary sclerosing cholangitis and advances in diagnosis and management.
 

Author(s): John E Eaton, Jayant A Talwalkar, Konstantinos N Lazaridis, Gregory J Gores, Keith D Lindor

Journal: Gastroenterology. 2013 Sep;145(3):521-36.

 

Primary sclerosing cholangitis (PSC), first described in the mid-1850s, is a complex liver disease that is heterogeneous in its presentation. PSC is characterized by chronic cholestasis associated with chronic inflammation of the biliary epithelium, resulting in multifocal bile duct ...

Last Updated: 26 Aug 2013

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Safety and Efficacy Study of Ursodeoxycholic Acid Therapy in Pediatric Primary Sclerosing Cholangitis
 

Status: Recruiting

Condition Summary: Primary Sclerosing Cholangitis

 

Last Updated: 25 Aug 2014

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A Trial of BTT1023 in Patients With Primary Sclerosing Cholangitis
 

Status: Not yet recruiting

Condition Summary: Primary Sclerosing Cholangitis

 

Last Updated: 10 Sep 2014

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Last Updated: 3 Feb 2015

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