P011 Clinical Outcomes of Primary Sclerosing Cholangitis in IBD Patients: A Single Center Experience

BACKGROUND: Primary sclerosing cholangitis (PSC) is a chronic and progressive cholestatic disease associated with Inflammatory Bowel Diseases (IBD) in 70% of cases. When combined, they present as one distinct phenotypical disease referred as PSC-IBD with milder clinical course, despite of more extensive colonic involvement and an increased risk of malignancies. The aim of this study was to assess the prevalence, characteristic features and long-term complications of PSC-IBD patients from a quaternary hospital in Brazil. METHODS: We retrospectively reviewed all PSC-IBD patients registered and followed-up in our center between January 2000 and July 2019. Conventional colonoscopic and histological criteria were used to diagnose IBD, whereas radiographic or histological criteria for PSC. Medical records were abstracted for the following data: demographic characteristics; duration, extent, and treatment of IBD; duration, extent, and treatment of PSC; colorectal neoplasia (dysplasia and cancer); surgical treatment for IBD; liver transplantation and death. RESULTS: In total, 84 patients with concurrent PSC and IBD were identified. Of 84 cases, 74 (87%) had Ulcerative Colitis (UC) and 11 (13%) were diagnosed with Crohn’s disease (CD). There was a slight predominance of male, 46 (54%). The mean age at both IBD and PSC diagnosis was 31 and 36 years, respectively. Most of them were diagnosed with IBD prior to PSC. The mean IBD duration was 15 years and 10 years in PSC subjects. The prevalence of pancolitis in UC patients was 80%. All CD cases presented ileocolonic involvement. Present or past smoking history was observed in 4 patients (4.7%). Aminosalicylates were the most common treatment used for IBD in 54 patients (64.3%). 15 of them (18%) were on monotherapy with immunobiologics and 7 patients (8.3%) were on combotherapy. Only 1 subject was on anti-integrin. In our cohort, 55 cases (66.7%) were treated with deoxycholic acid. Colorectal neoplasia and cholangiocarcinoma was observed in 6 (7%) and 2 patients (2.3%), respectively. Colorectal dysplasia was found in 12 subjects (22%) on a routine colonoscopy. Overall, 18 patients (21.4%) required proctocolectomy and 9 cases (10.7%) have undergone orthotopic liver transplant. There were 12 deaths, mainly PSC and cancer-related. CONCLUSION(S): This is the largest cohort of concomitant PSC and IBD patients in Latin America. In our center, we have found higher rates of extensive IBD although few of them were on biologics. The present data on cancer and dysplasia are consistent with reports in the literature.