HERACLIS-PSC: Current characteristics of primary sclerosing cholangitis with or without inflammatory bowel disease in Greece–a national multicenter cohort study

BACKGROUND: Studies from northern Europe indicate that patients with primary sclerosing cholangitis (PSC) are predominantly males and often have ulcerative colitis (UC) and increased risk of colorectal cancer (CRC). AIM: To evaluate the epidemiological and clinical features and natural history of PSC in Greece, a southern European country. METHODS: In the HERACLIS-PSC study, consecutive patients with PSC-IBD followed at Greek inflammatory bowel disease (IBD) centers were included and compared to data from consecutive patients with PSC-only followed at a Greek liver center. RESULTS: Among PSC-IBD patients (n=112, males:56%), 62.5% had UC (pancolitis:76%), 35.7% Crohn's disease and 1.8% indeterminate colitis. PSC was diagnosed at a younger age in PSC-IBD than PSC-only patients (n=39; males:54%) (32±15 vs 40±15 years; p=0.007), while the two groups did not differ significantly in gender, extrahepatic bile duct involvement, or development of cirrhosis, cholangiocarcinoma or CRC, although CRC occurred only in PSC-IBD (n=8). Transplant-free survival was higher in PSC-IBD than PSC-only (5-/10-year:95%/87% vs 85%/77%; p=0.021), while older age at PSC diagnosis and cirrhosis development were independently associated with lower transplant-free survival. CONCLUSION: In Greece, PSC-IBD patients are usually males with UC, in whom PSC is diagnosed at a younger age demonstrating better transplant-free survival, compared to PSC-only.