Primary sclerosing cholangitis.

In spite of an improved understanding of the etiology of primary sclerosing cholangitis, which supports the use of immunosuppressive therapy with steroids and azathioprine, these pharmacologic manipulations have not altered the ultimate outcome of the disease. Drainage remains the most accepted mode of therapy when possible. Recent advances in diagnosis with ERCP and treatment with transhepatic biliary drainage may change the time-honored surgical approach to this disease as these techniques become more widely available. Until then, however, proper surgical management depends upon a high index of suspicion at the time of laparotomy so that irrevocable damage to the biliary tree will not be done prior to the establishment of the correct diagnosis. Simple drainage of the biliary tree will provide symptomatic relief in some patients; unfortunately, most patients will succumb to progressive biliary cirrhosis or sepsis in spite of all treatment.