P231 Epidemiological profile and long-term outcomes from a large PSC-IBD cohort

A.E. Caon, C. Martins, M. Cruz, L. Leite Barros, M. Azevedo, N. Queiroz, A. Carlos, L. Milane, J. Oba, M. Braga, A. Sipahi, A. Damião, E. Cançado

1Department of Gastroenterology and Hepatology, University of São Paulo School of Medicine, São Paulo, Brazil


Primary sclerosing cholangitis (PSC) is a chronic and progressive cholestatic disease associated with inflammatory bowel diseases (IBD) in 70% of cases.1 When combined, they present as one distinct phenotypical disease referred to as PSC-IBD with milder clinical course, despite more extensive colonic involvement and an increased risk of malignancies. The aim of this study was to assess clinical features and long-term outcomes of PSC-IBD patients from a quaternary hospital in Brazil.


We retrospectively reviewed all PSC-IBD patients registered and followed-up in our outpatient clinic 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.


In total, 92 patients with concurrent PSC and IBD were identified. Of 92 cases, 80 (87%) had ulcerative colitis (UC) and 12 (13%) were diagnosed with Crohn’s disease (CD). There was a slight predominance of male, 51 (55%). The mean age at both IBD and PSC diagnosis was 31 and 35 years, respectively. Most of them were diagnosed with IBD prior to PSC (53%). The mean IBD duration was 15 years and 10 years in PSC subjects. The prevalence of pancolitis in UC patients was 91%. All CD cases presented ileocolonic involvement. Present or past smoking history was reported in 6% of the cohort. Aminosalicylates were the most common treatment used for IBD in 59 patients (64%). 15 of them (16%) were on monotherapy with immunobiologics and 7 patients (8%) were on combotherapy. Only 3 subjects were on anti-integrin. In our cohort, 61 cases (66%) were treated with ursodeoxycholic acid. Colorectal neoplasia and cholangiocarcinoma were observed in 6 (7%) and 3 patients (3.3%), respectively. Colorectal dysplasia was found in 20 subjects (22%) on a routine colonoscopy. Overall, 17 patients (19%) required proctocolectomy and 11 cases (12%) have undergone orthotopic liver transplant. There were 12 deaths, mainly PSC and cancer-related.


Herein we present the largest cohort of concomitant PSC and IBD in Latin America. In our centre, we have found higher rates of extensive IBD, although few of them required colectomy. The present data on cancer and dysplasia are consistent with reports in the literature.


Karlsen TH, Folseraas T, Thorburn D, Vesterhus M. Primary sclerosing cholangitis - a comprehensive review. J Hepatol. 2017;67(6):1298–1323.