Non-IBD immunological diseases are a risk factor for reduced survival in PSC
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 be sides IBD and AIH in a...
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| Main Authors: | , , , , , , , |
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| Format: | Article (Journal) |
| Language: | English |
| Published: |
2012
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| In: |
Liver international
Year: 2013, Volume: 33, Issue: 1, Pages: 86-93 |
| ISSN: | 1478-3231 |
| DOI: | 10.1111/liv.12028 |
| Online Access: | Verlag, Volltext: http://dx.doi.org/10.1111/liv.12028 Verlag, Volltext: https://onlinelibrary.wiley.com/doi/abs/10.1111/liv.12028 
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| Author Notes: | Christian Rupp, Anne Mummelthei, Peter Sauer, Karl H. Weiss, Peter Schirmacher, Adolf Stiehl, Wolfgang Stremmel and Daniel N. Gotthardt |
MARC
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| 245 | 1 | 0 | |a Non-IBD immunological diseases are a risk factor for reduced survival in PSC |c Christian Rupp, Anne Mummelthei, Peter Sauer, Karl H. Weiss, Peter Schirmacher, Adolf Stiehl, Wolfgang Stremmel and Daniel N. Gotthardt |
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| 520 | |a 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 be sides 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 ou r 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 repre sent 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 especial ly 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. | ||
| 650 | 4 | |a autoimmune liver disease | |
| 650 | 4 | |a biliary tract | |
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| 650 | 4 | |a Primary sclerosing cholangitis | |
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