HERACLIS-PSC: Current characteristics of primary sclerosing cholangitis with or without inflammatory bowel disease in...

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).

To evaluate the epidemiological and clinical features and natural history of PSC in Greece, a southern European country.

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.

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.

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.