Crohn’s disease behavior and location is altered when associated with primary sclerosing cholangitis

Background: Up to 3.4% of Crohn’s disease (CD) patients will be diagnosed with concomitant primary sclerosing cholangitis (PSC). Despite the worldwide increase incidence of CD, data on the clinical characteristics of PSC-CD patients are scarce. Objectives: To clinically characterize CD in patients who have concomitant PSC. Methods: A retrospective case-control analysis was conducted with 18 CD patients with concomitant PSC who attended the Inflammatory Bowel Disease Center at the Tel Aviv Sourasky Medical Center between 2011–2014 (PSC-CD patients). They were matched by age, gender, and disease duration to 90 control patients (those with CD who did not have concomitant PSC). Disease phenotype (according to the Montreal classification), demographics, and clinical data were compared in the two groups. Results: PSC-CD patients were characterized by a disease that was more frequently limited to the colon (L2) (50% vs. 16%, P = 0.004) and by a non-stricturing and non-penetrating inflammatory phenotype (83% vs. 33%, P = 0.0001) compared to controls who had an increased prevalence of the penetrating phenotype (B3) (6% vs. 33%, P < 0.05). Use of 5-aminosalicylic acid agents as a single therapy was significantly more prevalent among PSC-CD patients than in controls (39% vs. 7%, P < 0.005). In contrast, biologic therapy was significantly less common among PSC-CD patients compared to controls (17% vs. 52%, P = 0.0086). Conclusions: Patients with PSC-CD are clinically distinct from patients with isolated CD, and are characterized by predominant colonic involvement and an inflammatory, non-stricturing and non-penetrating phenotype.