BACKGROUND: Primary sclerosing cholangitis is a chronic cholestatic liver disease. An immune aetiology is suggested by associations between PSC and inflammatory bowel disease. Data on concomitant prevalence of other immune-mediated diseases is limited. AIM: To assess the prevalence of concomitant immune-mediated diseases and the impact on disease outcome in PSC. METHODS: We included 241 patients and retrospectively reviewed medical charts. RESULTS: Altogether 172 (71.4%) patients had concomitant immune-mediated disease, including IBD (149, 61.8%), autoimmune hepatitis (15, 6.2%) and other immune-mediated diseases (47, 19.5%). Thirty nine patients (22.7%) had more than one immune-mediated disease other than PSC. Most frequent extrahepatic non-IBD immune-mediated diseases were sarcoidosis, thyroid disease, and type I diabetes mellitus. Age at PSC diagnosis was lower in patients with IBD. In patients with other immune-mediated diseases than autoimmune hepatitis or IBD, age at PSC diagnosis was higher. Younger age at diagnosis and concomitant IBD related to longer survival till death or liver transplantation. CONCLUSIONS: In a large PSC population, a high prevalence of concomitant immune-mediated diseases was found. IBD occurred more often in early-acquired PSC, and the other immune-mediated diseases more often in later-acquired PSC. No effect on outcome was found for non-IBD immune mediated disease.
BACKGROUND:Primary sclerosing cholangitis is a chronic cholestatic liver disease. An immune aetiology is suggested by associations between PSC and inflammatory bowel disease. Data on concomitant prevalence of other immune-mediated diseases is limited. AIM: To assess the prevalence of concomitant immune-mediated diseases and the impact on disease outcome in PSC. METHODS: We included 241 patients and retrospectively reviewed medical charts. RESULTS: Altogether 172 (71.4%) patients had concomitant immune-mediated disease, including IBD (149, 61.8%), autoimmune hepatitis (15, 6.2%) and other immune-mediated diseases (47, 19.5%). Thirty nine patients (22.7%) had more than one immune-mediated disease other than PSC. Most frequent extrahepatic non-IBD immune-mediated diseases were sarcoidosis, thyroid disease, and type I diabetes mellitus. Age at PSC diagnosis was lower in patients with IBD. In patients with other immune-mediated diseases than autoimmune hepatitis or IBD, age at PSC diagnosis was higher. Younger age at diagnosis and concomitant IBD related to longer survival till death or liver transplantation. CONCLUSIONS: In a large PSC population, a high prevalence of concomitant immune-mediated diseases was found. IBD occurred more often in early-acquired PSC, and the other immune-mediated diseases more often in later-acquired PSC. No effect on outcome was found for non-IBD immune mediated disease.
Authors: Christian Rupp; Alexander Rössler; Taotao Zhou; Conrad Rauber; Kilian Friedrich; Andreas Wannhoff; Karl-Heinz Weiss; Peter Sauer; Peter Schirmacher; Caner Süsal; Wolfgang Stremmel; Daniel N Gotthardt Journal: United European Gastroenterol J Date: 2017-06-17 Impact factor: 4.623
Authors: Rudi Alberts; Elisabeth M G de Vries; Elizabeth C Goode; Xiaojun Jiang; Fotis Sampaziotis; Krista Rombouts; Katrin Böttcher; Trine Folseraas; Tobias J Weismüller; Andrew L Mason; Weiwei Wang; Graeme Alexander; Domenico Alvaro; Annika Bergquist; Niklas K Björkström; Ulrich Beuers; Einar Björnsson; Kirsten Muri Boberg; Christopher L Bowlus; Maria C Bragazzi; Marco Carbone; Olivier Chazouillères; Angela Cheung; Georgios Dalekos; John Eaton; Bertus Eksteen; David Ellinghaus; Martti Färkkilä; Eleonora A M Festen; Annarosa Floreani; Irene Franceschet; Daniel Nils Gotthardt; Gideon M Hirschfield; B van Hoek; Kristian Holm; Simon Hohenester; Johannes Roksund Hov; Floris Imhann; Pietro Invernizzi; Brian D Juran; Henrike Lenzen; Wolfgang Lieb; Jimmy Z Liu; Hanns-Ulrich Marschall; Marco Marzioni; Espen Melum; Piotr Milkiewicz; Tobias Müller; Albert Pares; Christian Rupp; Christian Rust; Richard N Sandford; Christoph Schramm; Stefan Schreiber; Erik Schrumpf; Mark S Silverberg; Brijesh Srivastava; Martina Sterneck; Andreas Teufel; Ludovic Vallier; Joanne Verheij; Arnau Vich Vila; Boudewijn de Vries; Kalliopi Zachou; Roger W Chapman; Michael P Manns; Massimo Pinzani; Simon M Rushbrook; Konstantinos N Lazaridis; Andre Franke; Carl A Anderson; Tom H Karlsen; Cyriel Y Ponsioen; Rinse K Weersma Journal: Gut Date: 2017-08-04 Impact factor: 23.059
Authors: Phil Meister; Christian Steinke-Ramming; Mechthild Beste; Henrike Lenzen; Guido Gerken; Ali Canbay; Christoph Jochum Journal: Diseases Date: 2020-06-12