Non‐IBD immunological diseases are a risk factor for reduced survival in PSC. (14th November 2012)

• Record Type: Journal Article
• Authors: Rupp, Christian; Mummelthei, Anne; Sauer, Peter; Weiss, Karl H.; Schirmacher, Peter; Stiehl, Adolf; Stremmel, Wolfgang; Gotthardt, Daniel N.
• Is Part Of: Liver international. Volume 33:Number 1(2013:Jan.)
• Page Start: 86
• Page End: 93
• Publication Date: 2012-11-14
• Subjects: Liver -- Periodicals; Liver -- Diseases -- Periodicals; 616.362
• DOI: 10.1111/liv.12028
• Languages: English
• ISSNs: 1478-3223

Abstract

<abstract abstract-type="main" id="liv12028-abs-0001"> <title>Abstract</title> <sec id="liv12028-sec-0001" sec-type="section"> <title>Background</title> <p>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).</p> </sec> <sec id="liv12028-sec-0002" sec-type="section"> <title>Aim</title> <p>We evaluated the presence of IDs besides IBD and AIH in a cohort of PSC patients, and its association with clinical outcome.</p> </sec> <sec id="liv12028-sec-0003" sec-type="section"> <title>Methods</title> <p>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.</p> </sec> <sec id="liv12028-sec-0004" sec-type="section"> <title>Results</title> <p>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, <italic>P</italic> = 0.012). Further subgroup analysis revealed a significantly reduced survival especially in patients with concomitant IMID (<italic>P</italic> = 0.017).</p> </sec> <sec id="liv12028-sec-0005" sec-type="section"> <title>Conclusion</title> <p>Patients with concomitant IDs, especially IMID, are a clinically important subgroup of PSC patients. This significant phenotype warrants further genetic and immunological studies.</p> </sec> </abstract>