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).
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