PSC Literature Logo

  Primary Sclerosing Cholangitis Literature  

Introduction

This site provides a compilation of www resources and scientific literature on primary sclerosing cholangitis (PSC), a chronic cholestatic liver disease characterized by periductal inflammation of both intrahepatic and extrahepatic bile ducts. Progressive loss of bile ducts impairs bile flow, ultimately resulting in cirrhosis. Males are affected by PSC more often than females (2:1), and the disease is often accompanied by inflammatory bowel disease; mostly ulcerative colitis and sometimes Crohn's disease. The exact causes of PSC are unknown, but it is thought that the disease might be triggered by as yet unidentified environmental factor(s) in genetically susceptible individuals.

The genetic basis of PSC is likely to be complex; certain of the genes determining susceptibility are thought to reside in the major histocompatibility complex - a genetic region of central importance in a number of autoimmune diseases. Because PSC is often associated with inflammatory bowel disease, the complex genetic basis of ulcerative colitis and Crohn's disease may be relevant to understanding how PSC is initiated and sustained.

The National Institute of Diabetes and Digestive and Kidney Diseases, the Office of Rare Diseases, and the Morgan Foundation jointly sponsored a Primary Sclerosing Cholangitis Conference in Bethesda, MD, September 19-20, 2005. The aims of the meeting were to stimulate clinical and basic research interest in PSC and identify gaps in knowledge and challenges for medical research in this disease:

LaRusso NF, Shneider BL, Black D, Gores GJ, James SP, Doo E, Hoofnagle JH 2006 Primary sclerosing cholangitis: summary of a workshop. Hepatology 44: 746-764

The European Association for the Study of the Liver (EASL) held a Monothematic Conference on “Primary Sclerosing Cholangitis” in Oslo, Norway June 21-23, 2009. Central questions that were addressed at this conference included:

·         Epidemiology and natural history of PSC

·         Pathogenesis of PSC

·         Overlap syndrome and autoimmunity in PSC

·         Malignancies in PSC: pathogenesis and treatment

·         Transplantation in PSC including recurrence

The Morgan Foundation has established a research initiative entitled STOPSC: 'Studies of Primary Sclerosing Cholangitis'. STOPSC is a collaborative effort between centers in the United States and Canada whose primary objective is to collect and analyze information required to understand the etiology, pathogenesis, and treatment of primary sclerosing cholangitis (PSC). Researchers who are interested in learning more about STOPSC should contact the STOPSC Data Coordinating Center (see About STOPSC).

This site is associated with PSC Partners Seeking a Cure (www.pscpartners.org), a 501(c)3 nonprofit foundation that endeavors to find a cure for Primary Sclerosing Cholangitis. Please consider a donation to help us conquer this devastating disease. For more information about how to make a tax-deductible donation, please visit PSC Partners Seeking a Cure (www.pscpartners.org).

Working together to provide research, education, and support for people affected by Primary Sclerosing Cholangitis

If you are a PSC researcher seeking grant support, please consult the PSC Partners Seeking a Cure: Request for Proposals. To see a list of previous grant winners through 2013 visit PSC Partners Seeking a Cure: Previous Grant Awards.

 

www.pscpartners.org
contactus@pscpartners.org

Last Update: 2014-01-09