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Review
. 2008 Jun 7;14(21):3350-9.
doi: 10.3748/wjg.14.3350.

Etiopathogenesis of primary sclerosing cholangitis

Roger Chapman  1 , Sue Cullen
Affiliations
Review

Etiopathogenesis of primary sclerosing cholangitis

Roger Chapman et al. World J Gastroenterol. .

Abstract

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown etiology but lymphocytic portal tract infiltration is suggestive of an immune-mediated basis for this disease. Associations with inflammatory bowel disease (IBD) especially ulcerative colitis (UC), and with particular autoimmune diseases, as well as the genetic associations further suggest PSC may be an immune-mediated disease. The immunogenetics of PSC have been the subject of active research and several HLA and non-HLA associated genes have been implicated in the development of the disease. Lymphocytes derived from the inflamed gut may enter the liver via the enterohepatic circulation to cause hepatic disease. PSC may be triggered in genetically susceptible individuals by infections or toxins entering the portal circulation through a permeable colon and hence evoking an abnormal immune response.

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Figures

Figure 1
Figure 1
Cholangiogram showing beading and dilatation of the intra and extra hepatic bile ducts- the diagnostic features of primary sclerosing cholangitis (PSC).
Figure 2
Figure 2
Vierling’s hypothesis of the pathogenesis of primary sclerosing cholangitis[72].
See this image and copyright information in PMC

References

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