Portincasa P, Vacca M, Moschetta A, Petruzzelli M, Palasciano G, van Erpecum KJ, van Berge-Henegouwen GP. Primary sclerosing cholangitis: Updates in diagnosis and therapy. World J Gastroenterol 2005; 11(1): 7-16 [PMID: 15609388 DOI: 10.3748/wjg.v11.i1.7]
Corresponding Author of This Article
Professor Piero Portincasa, M.D., PhD, Professor of Internal Medicine, Section of Internal Medicine, Department of Internal and Public Medicine (DIMIMP), University Medical School of Bari, P.zza G. Cesare 11, 70124 Bari, Italy. p.portincasa@semeiotica.uniba.it
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Review
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World J Gastroenterol. Jan 7, 2005; 11(1): 7-16 Published online Jan 7, 2005. doi: 10.3748/wjg.v11.i1.7
Primary sclerosing cholangitis: Updates in diagnosis and therapy
Piero Portincasa, Michele Vacca, Antonio Moschetta, Michele Petruzzelli, Giuseppe Palasciano, Karel J. van Erpecum, Gerard P. van Berge-Henegouwen
Piero Portincasa, Michele Vacca, Antonio Moschetta, Michele Petruzzelli, Giuseppe Palasciano, Section of Internal Medicine, Department of Internal Medicine and Public Medicine (DIMIMP), University Medical School, Bari, Italy
Karel J. van Erpecum, Gerard P. van Berge-Henegouwen, Department of Gastroenterology and Hepatology, University Hospital Utrecht, The Netherlands
Author contributions: All authors contributed equally to the work.
Correspondence to: Professor Piero Portincasa, M.D., PhD, Professor of Internal Medicine, Section of Internal Medicine, Department of Internal and Public Medicine (DIMIMP), University Medical School of Bari, P.zza G. Cesare 11, 70124 Bari, Italy. p.portincasa@semeiotica.uniba.it
Telephone: +390805478227 Fax: +390805478232
Received: May 25, 2004 Revised: May 28, 2004 Accepted: July 17, 2004 Published online: January 7, 2005
Abstract
Primary sclerosing cholangitis (PSC) is a chronic cholestatic syndrome of unknown origin mostly found in males, and characterized by diffuse inflammation and fibrosis of both intra- and extra-hepatic bile ducts. So far, PSC is considered as an autoimmune hepatobiliary disease. In most cases the progression of PSC towards liver cirrhosis and liver failure is slow but irreversible, and liver transplantation is currently the only definitive treatment. In recent years, PSC has been an area of active research worldwide with great interest in etiology, pathogenesis, diagnosis, and therapeutic options such as hydrophilic ursodeoxycholic acid and immunosuppressive agent tacrolimus. Recent updates on clinical and therapeutic aspects of PSC are discussed in the present review.
