Abstract

Background: Cholangiocarcinoma (CC) complicates in 10 20% of the patients with dominant stricture (DS) in patients with primary sclerosing cholangitis (PSC) related with inflammatory bowel diseases (IBD). Methods: We investigated 2200 patients with IBD for PSC with DS. Results: PSC was diagnosed in 41 pts (1.6%). Of the 41 pts with PSC, DS was diagnosed in 14 (34%); 64% were male, and followed for a mean of 4.86 yrs (0 13 yrs). Main finding was pruritis alone in 64% pts with DS as in 15% without DS (p = 0.003). The diagnose date of both IBD and PSC was younger in pts with DS than without DS (36.9 yrs vs. 38.1 and 40.2 yrs vs. 41.1 yrs; p > 0.05, respectively). Only current ALP and GGT levels were differ between the groups (p: 0.003 and p: 0.001, respectively). In this study, ERCP performed in 22 pts with PSC and was the main diagnostic tool for the questioning the occurence of DS (p = 0.011), besides CT, MRI and liver bx. ERCP usually performed more than once. EST and baloon dilation performed in all pts with stenting in 9 pts. Brush cytology performed only in 3 pts with strong suspicious for CC and benign pathology reported. IBD disease duration was longer in pts without DS than with DS (10.4 yrs vs. 8.1 yrs, p > 0.05). During the follow-up, 2 patients dead due to the causes other than CC; one due to endstage liver disease, and one had back wash ileitis complicated with colon malignancy and later died. Liver transplantation performed twice in one patient and still alive and in a good condition. At the same time, we evaluated our last 5-years pathology records for CC in a different study, and found 19 cases. None was related with our IBD or PSC-IBD group. Of the 19, colonoscopy performed in 5, and showed no IBD on biopsy. Conclusions: In our series, dominant strictures with PSC related with IBD showed a relatively benign nature differently from the current literature.

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