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Large-duct PSC, Crohn’s disease patients fare better than other PSC groups

Patients with large-duct primary sclerosing cholangitis and Crohn’s disease had less aggressive liver disease and better survival and transplant outcomes compared with patients with PSC without inflammatory bowel disease or with ulcerative colitis, according to retrospective study findings.

“Since Crohn’s disease is less often present in PSC patients than UC, few data are available on the characteristics of PSC in patients with concomitant [Crohn’s disease],” the researchers wrote. “We analyzed the disease evolution of our patients with PSC and [Crohn’s disease] considering small- and large-duct PSC separately in comparison with PSC patients with UC, [inflammatory bowel disease unclassified] or without [inflammatory bowel disease].

Researchers analyzed electronic health records of 240 patients from the database at Leuven University Hospital in Belgium diagnosed with PSC between 1975 and 2012. Primary and secondary endpoints included long-term survival without having to undergo liver transplantation (LT), the number of LT and liver-related mortality.

During a median follow-up of 12 years, 63% of patients had IBD. Of these, 105 were diagnosed with UC, 32 had Crohn’s disease, and 14 had an unclassified type of IBD.

Small-duct PSC was found in 28% of patients with PSC and Crohn’s disease vs. 3% of patients with PSC and UC. Patients with small-duct PSC had better survival rates compared with large-duct PSC. In these patients, none developed cholangiocarcinoma or died of a liver-related event. Three patients, however, developed colorectal cancer.

In patients with large-duct PSC, liver disease was less progressive compared with patients with small-duct PSC, indicating more favorable outcomes in patients with Crohn’s disease. Only one patient underwent LT compared with 28% of patients with all other PSC groups (P ≤ .002). In addition, there were no observed liver-related deaths compared with 7% in all other PSC groups.

“The liver disease of large-duct PSC patients with Crohn’s disease is less progressive than in those with UC or without IBD, resulting in a lower need for LT and in a rarity of liver-related deaths or complications,” the researchers concluded. – by Melinda Stevens

Disclosure: Fevery reports no relevant financial disclosures. Please see the full study for a list of all other authors’ relevant financial disclosures.