Non-IBD immunological diseases are a risk factor for reduced survival in PSC
Article first published online: 14 NOV 2012
© 2012 John Wiley & Sons A/S
Volume 33, Issue 1, pages 86–93, January 2013
How to Cite
Rupp, C., Mummelthei, A., Sauer, P., Weiss, K. H., Schirmacher, P., Stiehl, A., Stremmel, W. and Gotthardt, D. N. (2013), Non-IBD immunological diseases are a risk factor for reduced survival in PSC. Liver International, 33: 86–93. doi: 10.1111/liv.12028
- Issue published online: 10 DEC 2012
- Article first published online: 14 NOV 2012
- Accepted manuscript online: 12 OCT 2012 04:45AM EST
- Manuscript Accepted: 13 AUG 2012
- Manuscript Received: 29 FEB 2012
- autoimmune liver disease;
- biliary tract;
- cholestatic liver disease;
- Primary sclerosing cholangitis
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease. It is known to be associated with immunological diseases (IDs), such as inflammatory bowel disease (IBD) and autoimmune hepatitis (AIH).
We evaluated the presence of IDs besides IBD and AIH in a cohort of PSC patients, and its association with clinical outcome.
This is a prospective cohort study of 195 PSC patients that were evaluated over the period 1987–2010 in our tertiary care centre. The presence of ID was determined using a retrospective chart review. IDs were subclassified into autoimmune disease (AID) and immune-mediated inflammatory disease (IMID), according to current guidelines.
Twenty-seven of 195 (13.8%) PSC patients had at least one additional ID other than IBD (70%) or AIH (5%). The most frequent AIDs were autoimmune thyroiditis (2.6%) and diabetes mellitus type 1 (2.1%). The most frequent IMIDs were psoriasis (3.6%) and sarcoidosis (2.1%). After more than 20 years of follow-up, concomitant IDs represent an independent risk factor for reduced transplantation-free survival in patients with PSC (mean: 8.9 years vs. 16.3 years, P = 0.012). Further subgroup analysis revealed a significantly reduced survival especially in patients with concomitant IMID (P = 0.017).
Patients with concomitant IDs, especially IMID, are a clinically important subgroup of PSC patients. This significant phenotype warrants further genetic and immunological studies.