Abstract

Ulcerative colitis (UC) associated with primary sclerosing cholangitis (PSC) is associated with pancolitis and clinically mild disease. The aim of the study was to study the association between the severity of PSC and clinical outcome of UC, comparing the course of UC in patients with PSC not needing orthotopic liver transplantation (OLT) and those requiring LT. A total of 167 patients with PSC and UC seen at the Cleveland Clinic between 1985 and 2011 were included in this study. Clinical and demographic variables were obtained regarding the outcome of UC in patients with and without OLT for underlying UC. Patients were followed-up until OLT or the date of the last clinical visit. Logistic regression model was constructed by including variables known at UC or PSC diagnosis for the risk of colectomy. Patients with PSC/UC were divided into two groups: 81 did not need OLT (no-OLT) and 86 were transplanted (OLT). 81 (94.2%) of patients in the OLT group had pancolitis in contrast to 72 (88.9%) in the no-OLT group. (p=0.005) There were no significant differences concerning duration of UC between the two groups, while the duration of PSC was longer in the OLT group [median of 13.5 (interquartile range 10, 20) vs. 11(7.5, 17), p=0.01] than the no-OLT group. The median follow-up was a little higher for the OLT group (13.5 vs. 11 years, p=0.009). The OLT group had significantly more clinically quiescent UC compared with the no-OLT group. (59 (68.6%) vs. 12 (14.8%), p<0.001) The OLT group had fewer UC flare-ups [3 (2, 6) vs. 0 (0, 1), p<0.001) and required less use of azathioprine [(1 (1.2%) vs. 14 (17.3%), p=0.006]. There was an increased need for surgery in the no-OLT group [(61 (75.3%) vs. 23 (26.7%), p<0.001]. Colon carcinoma and dysplasia were also more frequent in the no-OLT group [(33 (40.7%) vs. 22 (25.6%), p=0.04]. On Cox regression analysis, OLT for PSC [Odds ratio (OR) 0.15 (95% confidence interval (C.I): 0.06 - 0.37, p<0.001] and higher Mayo risk score at diagnosis [OR 0.26, 95% CI: (0.13 - 0.52), p<0.001] independently protected against the risk of colectomy. Table 1 shows Cox regression analysis for the risk of colectomy. Cox regression analysis for the risk of colectomy Cox regression analysis for the risk of colectomy Severe PSC requiring OLT is associated with a milder course of UC (reduced disease activity and less use of azathioprine and surgery). Decreased disease activity is also associated with reduced incidence of dysplasia and colon carcinoma. The requirement for OLT and a higher Mayo PSC risk score at diagnosis appears to independently protect against colectomy.

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