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

• Verfasst von: Rupp, Christian [VerfasserIn]
• Verfasst von: Mummelthei, Anne [VerfasserIn]
• Verfasst von: Sauer, Peter [VerfasserIn]
• Verfasst von: Weiss, Karl Heinz [VerfasserIn]
• Verfasst von: Schirmacher, Peter [VerfasserIn]
• Verfasst von: Stiehl, Adolf [VerfasserIn]
• Verfasst von: Stremmel, Wolfgang [VerfasserIn]
• Verfasst von: Gotthardt, Daniel [VerfasserIn]
• Titel: Non-IBD immunological diseases are a risk factor for reduced survival in PSC
• Verf.angabe: Christian Rupp, Anne Mummelthei, Peter Sauer, Karl H. Weiss, Peter Schirmacher, Adolf Stiehl, Wolfgang Stremmel and Daniel N. Gotthardt
• Jahr: 2012
• Umfang: 8 S.
• Teil: volume:33
• Teil: year:2013
• Teil: number:1
• Teil: pages:86-93
• Teil: extent:8
• Fussnoten: Gesehen am 15.05.2018
• Titel Quelle: Enthalten in: Liver international
• Ort Quelle: Oxford : Wiley-Blackwell, 2003
• Jahr Quelle: 2013
• Band/Heft Quelle: 33(2013), 1, Seite 86-93
• ISSN Quelle: 1478-3231
• DOI: doi:10.1111/liv.12028
• Datenträger: Online-Ressource
• Sprache: eng
• Sach-SW: autoimmune liver disease
• Sach-SW: biliary tract
• Sach-SW: cholestatic liver disease
• Sach-SW: Primary sclerosing cholangitis
• Sach-SW: retinoid metabolism
• K10plus-PPN: 1575150492

Abstract

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.