Clinical Outcomes in Patients with Primary Sclerosing Cholangitis With and Without Inflammatory Bowel Disease.

[BACKGROUND] Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with inflammatory bowel disease (IBD) present in 60-80% of affected individuals. We aimed to investigate whether concurrent IBD worsens outcomes in PSC patients.

[METHODS] We conducted a retrospective cohort study using the TriNetX database to identify patients (≥ 18 years) with PSC. Patients were then divided into two groups: PSC with IBD (PSC-IBD cohort) and PSC without IBD (isolated PSC cohort). Propensity score matching (PSM) was used to control for covariates between both cohorts. The primary outcome was the risk of developing liver-related decompensation (combined ascites, hepatic encephalopathy, and variceal hemorrhage). Secondary outcomes included colorectal cancer (CRC), hepatobiliary malignancy, liver transplantation, and overall mortality. Cox proportional hazards models were used to calculate hazard ratios (HR) with 95% confidence intervals (CIs).

[RESULTS] We identified 6,690 patients with PSC, including 3,012 patients (45%) with isolated PSC and 3,678 patients (55%) with PSC-IBD. After PSM, 1,714 pairs were well-balanced. Over an average of 5-year follow-up, PSC-IBD patients had a higher risk of liver-related decompensation (HR 1.26, 95% CI 1.09-1.46, p = 0.001), cholangiocarcinoma (HR 1.38, 95%CI 1.08-1.76, p = 0.008), and liver transplantation (HR 1.50, 95%CI 1.28-1.78, p < 0.001) compared to those with isolated PSC, with no difference in overall mortality. CRC was more common in IBD-PSC patients (HR 3.91, 95%CI 2.46-5.21, p < 0.001). Subgroup analysis revealed that patients with ulcerative colitis had more severe liver disease than Crohn's disease patients.

[CONCLUSION] Concurrent IBD was associated with adverse clinical outcomes in patients with PSC, including an increased risk of liver-related decompensation and cholangiocarcinoma.