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

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...

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Bibliographic Details
Published in:Liver international 2013-01, Vol.33 (1), p.86-93
Main Authors: Rupp, Christian, Mummelthei, Anne, Sauer, Peter, Weiss, Karl H., Schirmacher, Peter, Stiehl, Adolf, Stremmel, Wolfgang, Gotthardt, Daniel N.
Format: Article
Language:English
Subjects:
Activation-induced cytidine deaminase
Adult
Autoimmune diseases
Autoimmune Diseases - mortality
autoimmune liver disease
biliary tract
Chi-Square Distribution
Cholagogues and Choleretics - therapeutic use
Cholangitis
Cholangitis, Sclerosing - mortality
Cholangitis, Sclerosing - therapy
cholestatic liver disease
Diabetes mellitus
Diabetes Mellitus, Type 1 - mortality
Disease-Free Survival
Female
Guidelines
Hepatitis
Humans
Immunological diseases
Inflammation - mortality
Inflammatory bowel diseases
Kaplan-Meier Estimate
Liver
Liver diseases
Liver Transplantation
Male
Multivariate Analysis
Prevalence
Primary sclerosing cholangitis
Proportional Hazards Models
Psoriasis
Psoriasis - mortality
Retrospective Studies
Reviews
Risk Assessment
Risk Factors
Sarcoidosis
Sarcoidosis - mortality
Survival
Tertiary Care Centers
Thyroiditis
Thyroiditis, Autoimmune - mortality
Time Factors
Ursodeoxycholic Acid - therapeutic use
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Summary: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.
ISSN:1478-3223
1478-3231
DOI:10.1111/liv.12028