Clinical and Prognostic Factors Associated With Inflammatory Bowel Disease in a Highly Admixed Population With Primary Sclerosing Cholangitis

Goals: To investigate the prevalence of inflammatory bowel disease (IBD) in a cohort of Brazilian patients with primary sclerosing cholangitis (PSC) and evaluate clinical and prognostic factors associated with concomitant IBD. Background: IBD is reported worldwide in 62% to 81% of patients with PSC, especially in males and north Europeans. Little is known about the association of IBD and PSC in population from multigenetic ethnic origin. Study: Data of PSC patients from the Brazilian Cholestasis Study Group database were retrospectively reviewed to compare demographic, clinical, laboratory, and transplant-free survival between those with and without IBD. Results: After exclusion of 59 (14%) participants with overlap syndrome with autoimmune hepatitis, 359 individuals with PSC were included {56% male, median age 44 [interquartile range (IQR): 33 to 54] y}. IBD was investigated in 298 (83%) participants and diagnosed in 217 (73%), including ulcerative colitis (83%), Crohn’s disease (13%), and indeterminate colitis (4%). Male sex frequency was similar in patients with and without IBD (58% vs. 47%, P =0.073). IBD was more frequently diagnosed in patients without obesity ( P =0.035), positive for antinuclear antibody ( P =0.006), and positive for anti-smooth muscle antibody ( P =0.046). IBD diagnosis occurred before, concomitant, or after PSC diagnosis in 59%, 22%, and 19% of cases, respectively. IBD was more frequently diagnosed before PSC in participants asymptomatic for liver disease ( P =0.017), without advanced liver disease ( P =0.017), before liver transplantation (LT) ( P <0.001), and positive for antinuclear antibody ( P =0.021). In a median follow-up of 69 months (IQR 31-124), LT occurred in 27.7% and cohort mortality was 11.4%. IBD was neither associated with the combined outcome of death or LT ( P =0.745) nor with transplant-free survival ( P =0.902). Conclusions: In Brazilian PSC patients, IBD frequency was similar to that reported in other populations but exhibited a balanced proportion between males and females. IBD diagnosis occurred before PSC in most patients, especially those without advanced liver disease. IBD presence was not associated with worse PSC prognosis.