Primary Sclerosing Cholangitis

 

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Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease characterized by fibrosis leading to progressive stricturing of the biliary system. It was first described in 1874, but more than a century later, fewer than 100 cases had been reported. However, in the last 25 years the development of endoscopic and radiologic techniques allowed the diagnosis of PSC to be made without resort to surgery or postmortem examinations. This led to the realization that PSC was much more common than previously thought. Since 1980, more than 1500 articles on PSC have been published.

What have we learned about this intriguing disease? Sadly, there are still more questions than answers. The etiopathogenesis is still unknown, although it is clear that any hypothesis has to explain the close association with male gender, nonsmoking status, and inflammatory bowel disease, which coexists in about 80% of patients in the northern hemisphere.

The natural history of the disease has become more clearly understood: PSC is a premalignant disease, and the majority of patients die from hepatobiliary or colonic cancer, although it is not possible to predict which patients are at risk of developing malignancy.

Specific medical treatment has not been established, but the results from liver transplantation have been comparable with other liver diseases. In this edition of Seminars in Liver Disease, I have been fortunate to be able commission the services of acknowledged experts in the field to review the many issues surrounding this fascinating disease. In addition to describing the state of the art, they have highlighted the many outstanding questions that need to be addressed by future research.