Large duct primary sclerosing cholangitis with or without inflammatory bowel disease in Saudi patients.

Background: Primary sclerosing cholangitis (PSC) is often associated with inflammatory bowel disease (IBD). We aimed to compare the clinical presentations and outcomes of PSC in patients with and without IBD in Saudi population.

Methods: The medical records of patients with PSC, diagnosed between 2009 and 2020, in a single referral center in Saudi Arabia were reviewed. Primary outcomes included liver-related clinical decompensations, while secondary outcomes encompassed hepatobiliary malignancies, liver-related mortality, and liver transplantation.

Results: Among 87 patients, 52 (59.8%) had PSC-IBD and 35 (40.2%) had PSC without IBD. PSC-IBD patients were significantly younger at diagnosis (35.4 ± 13.3 vs. 43.6 ± 12.8 years, P = 0.005). Non-IBD patients exhibited higher rates of diabetes (P = 0.045), hyperlipidemia (P = 0.022), and autoimmune hepatitis (P = 0.008). While clinical presentations were similar, non-IBD patients demonstrated more frequent cytopenia from splenomegaly (31.4% vs. 5.8%, P = 0.001) and hepatic decompensation (37.1% vs. 7.7%, P = 0.001). Bilirubin (P = 0.002) and aspartate aminotransferase (P = 0.042) levels were increased in non-IBD patients, while PSC-IBD patients had higher white blood cell (P = 0.021) and platelet counts (P < 0.001). Liver biopsies were more frequently performed in non-IBD patients (45.7% vs. 19.2%, P = 0.008). Overall mortality was 12.8%, with no difference in median survival time between groups (P = 0.782). Multivariate analysis identified age (hazard ratio [HR]: 1.048, P = 0.044) and MELD-Na (HR: 1.155, P = 0.016) as independent predictors of mortality.

Conclusion: In this Saudi cohort, non-IBD PSC patients were diagnosed later and presented with more comorbidities and hepatic decompensation. However, IBD status did not impact overall survival. Older age and higher MELD-Na scores at presentation were significant risk factors for mortality in PSC patients.