Non-IBD immunological diseases are a risk factor for reduced survival in PSC

Authors


Correspondence

Daniel Nils Gotthardt, Im Neuenheimer Feld 410, Heidelberg 69120, Germany

Tel: + 49-6221-56-39021

Fax: + 49-6221-56-5687

e-mail: Daniel.Gotthardt@med.uni-heidelberg.de

Abstract

Background

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).

Aim

We evaluated the presence of IDs besides IBD and AIH in a cohort of PSC patients, and its association with clinical outcome.

Methods

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.

Results

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).

Conclusion

Patients with concomitant IDs, especially IMID, are a clinically important subgroup of PSC patients. This significant phenotype warrants further genetic and immunological studies.

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