Evolving concepts in primary sclerosing cholangitis.

Abstract

Patients suffering from primary sclerosing cholangitis (PSC) show considerable differences regarding clinical manifestations (i.e. large duct versus small-duct PSC, presence or absence of concomitant inflammatory bowel disease), disease progression, risk for malignancy and response to therapy, raising the question whether PSC may represent a mixed bag of diseases of different aetiologies. The growing list of secondary causes and diseases 'mimicking' or even overlapping with PSC (e.g. IgG4-associated sclerosing cholangitis), which frequently causes problems in clear-cut discrimination from classic PSC and the emerging knowledge about potential disease modifier genes (e.g. variants of CFTR, TGR5 and MDR3) support such a conceptual view. In addition, PSC in children differs significantly from PSC in adults in several aspects resulting in distinct therapeutic concepts. From a clinical perspective, appropriate categorization and careful differential diagnosis are essential for the management of concerned patients. Therefore, the aim of the current review is to summarize current and evolving pathophysiological concepts and to provide up-to-date perspectives including future treatment strategies for PSC.

• Authors: Elisabeth Krones, Ivo Graziadei, Michael Trauner, Peter Fickert
• Journal: Liver international : official journal of the International Association for the Study of the Liver (Liver Int) Vol. 32 Issue 3 Pg. 352-69 (Mar 2012) ISSN: 1478-3231 [Electronic] United States
• PMID: 22097926 (Publication Type: Journal Article, Review)
• Copyright: © 2011 John Wiley & Sons A/S.

Chemical References

• Alkaline Phosphatase

Topics

• Adult
• Age Factors
• Alkaline Phosphatase (blood)
• Biopsy
• Child
• Cholangiography (methods)
• Cholangitis, Sclerosing (classification, complications, diagnosis, drug therapy, epidemiology, physiopathology)
• Diagnosis, Differential
• Humans
• Incidence
• Inflammatory Bowel Diseases (complications)
• Liver (pathology)
• Liver Transplantation (methods)
• Precancerous Conditions (pathology)
• Prevalence