Stephan R Vavricka1, Thomas Greuter2, Michael Scharl2, Gerassimos Mantzaris3, Ariella B Shitrit4, Rafal Filip5, Konstantinos Karmiris6, Christoph K Thoeringer7, Hubert Boldys8, Anne V Wewer9, Henit Yanai10, Cristina Flores11, Carsten Schmidt12, Revital Kariv13, Gerhard Rogler2, Jean-François Rahier14. 1. Division of Gastroenterology and Hepatology, University Hospital Zurich, Zurich, Switzerland Division of Gastroenterology and Hepatology, Triemli Hospital Zurich, Zurich, Switzerland. 2. Division of Gastroenterology and Hepatology, University Hospital Zurich, Zurich, Switzerland. 3. Department of Gastroenterology, Evangelismos Hospital, Athens, Greece. 4. Digestive Diseases Institute, Shaare Zedek Medical Center, Hebrew University Medical School, Jerusalem, Israel. 5. Department of Clinical Endoscopy, Institute of Rural Health, Lublin, Poland. 6. Department of Gastroenterology, Venizeleio General Hospital, Heraklion, Crete, Greece. 7. II. Medizinische Klinik und Poliklinik, Klinikum rechts der Isar, Technical University of Munich, Munich, Germany. 8. Department of Gastroenterology and Hepatology, Medical University of Silesia, Katowice, Poland. 9. Department of Pediatrics, Hvidovre University Hospital, Hvidovre, Denmark. 10. IBD Center, Department of Gastroenterology and Liver Diseases, Tel Aviv Medical Center, Tel Aviv, Israel, affiliated to the Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. 11. Gastroenterology Section, Porto Alegre Clinical Hospital, Federal University of Rio Grande do Sul, Porto Alegre, Brasil. 12. Department of Gastroenterology, University Hospital Jena, Jena, Germany. 13. Department of Gastroenterology and Liver Diseases, Tel Aviv Medical Center, Tel Aviv, Israel. 14. Department of Hepatogastroenterology, CHU Dinant Godinne, UCL Namur, Yvoir, Belgium.
Abstract
BACKGROUND: Cogan's syndrome (CSy) is a very rare autoimmune disorder, mainly affecting the inner ear and the eye, and is associated with inflammatory bowel disease (IBD). METHODS: This was a European Crohn's and Colitis Organisation (ECCO) retrospective observational study, performed as part of the CONFER project. A call to all ECCO members was made to report concomitant CSy and inflammatory bowel disease (IBD) cases. Clinical data were recorded in a standardized questionnaire. RESULTS: This international case series reports on 22 concomitant CSy-IBD cases from 14 large medical centres. Mean duration of IBD until diagnosis of CSy was 8.7 years (range 0.0-38.0) and mean age at CSy diagnosis was 44.6 years (range 9.0-67.0). Six patients had underlying ulcerative colitis (UC) and 16 had Crohn's disease. Eleven patients (50%) had active disease at CSy diagnosis. Sixteen patients were under IBD treatment at the time of CSy diagnosis, of whom 6 (37.5%) were on anti-tumour necrosis factor (TNF). Seven out of 10 patients, who were treated for CSy with immunomodulators (mostly with corticosteroids), demonstrated at least partial response. CONCLUSION: This is the largest CSy-IBD case series so far. Although CSy is considered to be an autoimmune disease and is associated with IBD, immunomodulatory IBD maintenance treatment and even anti-TNF therapy do not seem to prevent disease onset. Moreover, IBD disease activity does not seem to trigger CSy. However, vigilance may prompt early diagnosis and directed intervention with corticosteroids at inception may potentially hinder audiovestibular deterioration. Finally, vigilance and awareness may also offer a better setting to study the pathophysiological mechanisms of this rare but debilitating phenomenon.
BACKGROUND:Cogan's syndrome (CSy) is a very rare autoimmune disorder, mainly affecting the inner ear and the eye, and is associated with inflammatory bowel disease (IBD). METHODS: This was a European Crohn's and Colitis Organisation (ECCO) retrospective observational study, performed as part of the CONFER project. A call to all ECCO members was made to report concomitant CSy and inflammatory bowel disease (IBD) cases. Clinical data were recorded in a standardized questionnaire. RESULTS: This international case series reports on 22 concomitant CSy-IBD cases from 14 large medical centres. Mean duration of IBD until diagnosis of CSy was 8.7 years (range 0.0-38.0) and mean age at CSy diagnosis was 44.6 years (range 9.0-67.0). Six patients had underlying ulcerative colitis (UC) and 16 had Crohn's disease. Eleven patients (50%) had active disease at CSy diagnosis. Sixteen patients were under IBD treatment at the time of CSy diagnosis, of whom 6 (37.5%) were on anti-tumour necrosis factor (TNF). Seven out of 10 patients, who were treated for CSy with immunomodulators (mostly with corticosteroids), demonstrated at least partial response. CONCLUSION: This is the largest CSy-IBD case series so far. Although CSy is considered to be an autoimmune disease and is associated with IBD, immunomodulatory IBD maintenance treatment and even anti-TNF therapy do not seem to prevent disease onset. Moreover, IBD disease activity does not seem to trigger CSy. However, vigilance may prompt early diagnosis and directed intervention with corticosteroids at inception may potentially hinder audiovestibular deterioration. Finally, vigilance and awareness may also offer a better setting to study the pathophysiological mechanisms of this rare but debilitating phenomenon.
Authors: Fotios S Fousekis; Maria Saridi; Eleni Albani; Fady Daniel; Konstantinos H Katsanos; Ioannis G Kastanioudakis; Dimitrios K Christodoulou Journal: J Clin Med Res Date: 2018-06-27