作者: Craig Lammert , Raj Vuppalanchi
DOI: 10.1007/978-3-319-40908-5_12
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摘要: Primary sclerosing cholangitis (PSC) is a rare disease characterized by inflammation and fibrosis of the intra- extrahepatic bile ducts leading to biliary strictures, parenchymal fibrosis, subsequent progression cirrhosis liver failure. The often associated with concomitant inflammatory bowel (IBD) has varied phenotype progression. PSC an orphan disease, currently there no effective treatment other than transplantation. Fortunately, recent breakthroughs in understanding pathogenesis diseases have unraveled several new targets for PSC. In this chapter, we review current rationale behind novel therapeutic interventions. launch additional trials will bring us step closer treatments eventual cure