Recent Advance in Basic Science
Primary sclerosing cholangitis: recent advances
Marco Sciveres 1
1Epatologia Pediatrica e Trapianto di Fegato, ISMETT, University of Pittsburgh Medical Center Italy, Palermo
Primary sclerosing cholangitis is a progressive fibrosclerotic disease of the biliary tree. In the last decade several clinical variants were reported both in adults than in children. The description of small duct sclerosing cholangitis with its milder clinical course challenged the traditional concept of untreatable disease. Through the recent, discover of several genetic risk-loci and the future better comprehension of pathogenetic mechanisms novel targeted therapies would hopefully prevent the progression of disease to end stage liver damage and transplantation.