Immunologic diseases linked to poorer survival in PSC

Primary sclerosing cholangitis (PSC) patients with concomitant immunologic disorders (ID), have reduced transplantation-free survival compared with patients without, say the authors of a German study.

Patients with immune-mediated inflammatory disorders (IMID), such as psoriasis and sarcoidosis, were particularly at risk.

"Physicians should be aware of the presence of concomitant ID in patients with PSC to adequately treat them," say Daniel Nils Gotthardt (University Hospital of Heidelberg) and colleagues.

The study included 195 patients with PSC who were treated with ursodeoxycholic acid and endoscopic therapy between 1987 and 2010.

Overall, 132 also had inflammatory bowel disease (IBD), and 27 had concomitant ID other than IBD. Of these patients, 15 had an autoimmune disorder (AID) and 12 had IMID.

During follow up over a median time of 7.2 years, 26 patients underwent liver transplantation, and 16 patients died.

PSC patients with ID were significantly less likely to survive without transplant during up to 22 years follow up than patients without any ID (59.3 vs 81.5%). The median survival free of transplantation was 8.9 years for patients with any ID compared with 16.3 years without any ID.

However, multivariate analysis, including age at diagnosis, gender, Mayo risk score (MRS), and the presence of dominant stenosis, IBD, or non-IBD ID, showed that MRS was the only factor significantly associated with longer survival.

When the authors broke down IDs into IMID and AID, only IMID was significantly associated with lower transplantation-free survival compared with patients without ID (10.6 vs 41.7).

Interestingly, they did not find a significant association between IBD and reduced survival, which has been reported in previous studies.

"That an impact on survival was seen especially among patients with concomitant IMID may be a hint for a specific immunological background of PSC and may point to a new subgroup of patients with a more severe phenotype," say Nils Gotthardt and colleagues.

However, the reasons why IDs affect the transplantation-free survival of patients with PSC are unknown, they explain, adding that further research will be needed into the genetic and immunologic background of the disease.

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