Primary sclerosing cholangitis: outcome of patients undergoing restorative proctocolecetomy for ulcerative colitis

Purpose The prevalence of primary sclerosing cholangitis (PSC) among patients with ulcerative colitis needing proctocolectomy is about 12%. The study aim was to evaluate the progression of the liver disease after surgery. Methods PSC progression in 68 patients with UC after restorative proctocolectomy was evaluated after a median follow-up of 11 years (range 0 to 21). Magnetic resonance imaging (MRI) of the liver, histological examination of a core needle liver specimen, and liver function tests were used in addition to clinical history. Results Of the 68 patients, 30 participated in follow-up examinations. Ductal changes in MRI suggesting a diagnosis of PSC occurred in 21 (72%) of them. One carcinoma of the gallbladder was found in MRI. Histopathologic changes suggesting PSC were observable in 15 (50%) patients. Compared to stage in peroperative biopsies taken at proctocolectomy, PSC stage increased in four (13%) patients, decreased in 15 (50%), and remained unchanged in 11 (37%). Immunohistochemical staining for cytoceratin-7 in hepatocytes was positive in nine (30%) indicating cholestasis. After IPAA surgery, five patients underwent liver transplantation at 1, 1, 5, 6, and 11 years, respectively. Of the 68, six patients have, to date, developed cholangiocarcinoma. Conclusions Progression of PSC in patients with minor ductal changes at the time of restorative proctocolectomy is unlikely. Those patients with more advanced disease at surgery are at risk for disease progression and liver transplantation. We lack accurate diagnostic methods to detect premalignant changes of the biliary epithelium.