P275 Clinical characteristics, associated malignancies and management of primary sclerosing colangitis in inflammatory bowel disease patients: A Spanish nationwide study based on the ENEIDA registry

Abstract

Background: Primary sclerosing colangitis (PSC) is a chronic cholestasic liver disease usually associated with inflammatory bowel disease (IBD). An increased risk of malignancies (colorectal cancer and cholangiocarcinoma) has been reported in IBD patients with PSC. Our aim was to determine the clinical characteristics, associated malignancies and management of PSC in Spanish IBD patients. Methods: PSC patients with IBD were identified from the Spanish prospectively maintained ENEIDA registry of GETECCU. Additional data were collected using REDCap electronic data capture tool hosted at AEG (Asociación Española de Gastroenterología). Results: We identified 245 patients with PSC and IBD, 69% males, 96% Caucasian, 67% with ulcerative colitis, 29% Crohn's disease and 4% with unclassified colitis. Median age at diagnosis of PSC was 38 years (IQR 27-51). PSC was diagnosed after a median of 60 months (IQR 1-147) of IBD diagnosis, being PSC diagnosed before IBD in 12% of patients. Magnetic resonance cholangiopancreatography was the most common diagnostic technique used (77%). Liver biopsy was obtained in 72 patients (29%). Most of the patients (65%) were asymptomatic at PSC diagnosis. In symptomatic patients, pruritus was reported in 28 patients (11%), abdominal pain in 23 (9.4%), jaundice in 18 (7.3%), asthenia in 17 (6.9%) and cholangitis in 9 (3.7%). Phosphatase alkaline was permanently elevated in 53% of patients. IgG4 was positive in 20% of patients, ANA in 38%, SMA in 24% and AMA in 3.1%. Treatment with ursodeoxycholic acid was prescribed in 206 patients (84%) during a median of 70 months (IQR 36-130), with a mean dose of 14 ± 4mg/kg/d. Twenty-one patients (8.6%) required liver transplantation during follow-up, with post-transplantation complications in 9 of them (43%). Sixty-eight percent of patients followed a screening program with colonoscopy every 1-2 years, detecting 9 patients with high-grade dysplasia or adenocarcinoma (5.4%). Thirteen patients (5.3%) developed colorectal cancer, 85% after PSC diagnostic, and 7 patients (2.9%) were diagnosed with cholangiocarcinoma (3 intrahepatic, 4 extrahepatic). Surgery was performed in 2 patients with cholangiocarcinoma, 2 were endoscopically resected and 2 patients received chemotherapy and/or radiotherapy. Having a longer evolution of IBD was the only factor associated with the appearance of colorectal cancer in the multivariate analysis (p = 0.01), without finding any factor associated with cholangiocarcinoma development. Conclusions: In this nationwide study it is confirmed that the association of IBD and PSC is more common in males with ulcerative colitis, being PSC usually asymptomatic at diagnosis. Colorectal cancer is more frequent in patients with a longer evolution of IBD and after PSC diagnosis.