Cogan's Syndrome in Patients With Inflammatory Bowel Disease - a Case Series

Vavricka SR1, Greuter T2, Scharl M2, Mantzaris G3, Shitrit AB4, Filip R5, Karmiris K6, Thoeringer CK7, Boldys H8, Wewer AV9, Yanai H10, Flores C11, Schmidt C12, Kariv R13, Rogler G2, Rahier JF14. J Crohns Colitis. 2015 Jul 17. pii: jjv128. [Epub ahead of print]
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1Division of Gastroenterology and Hepatology, University Hospital Zurich, Zurich, Switzerland Division of Gastroenterology and Hepatology, Triemli Hospital Zurich, Zurich, Switzerland stephan.vavricka@triemli.zuerich.ch. 2Division of Gastroenterology and Hepatology, University Hospital Zurich, Zurich, Switzerland. 3Department of Gastroenterology, Evangelismos Hospital, Athens, Greece. 4Digestive Diseases Institute, Shaare Zedek Medical Center, Hebrew University Medical School, Jerusalem, Israel. 5Department of Clinical Endoscopy, Institute of Rural Health, Lublin, Poland. 6Department of Gastroenterology, Venizeleio General Hospital, Heraklion, Crete, Greece. 7II. Medizinische Klinik und Poliklinik, Klinikum rechts der Isar, Technical University of Munich, Munich, Germany. 8Department of Gastroenterology and Hepatology, Medical University of Silesia, Katowice, Poland. 9Department of Pediatrics, Hvidovre University Hospital, Hvidovre, Denmark. 10IBD 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. 11Gastroenterology Section, Porto Alegre Clinical Hospital, Federal University of Rio Grande do Sul, Porto Alegre, Brasil. 12Department of Gastroenterology, University Hospital Jena, Germany. 13Department of Gastroenterology and Liver Diseases, Tel Aviv Medical Center, Tel Aviv, Isreal. 14Department of Hepatogastroenterology, CHU Dinant Godinne, UCL Namur, Yvoir, Belgium.


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).

MATERIALS AND METHODS: This was a European Crohn's and Colitis Organization (ECCO) retrospective observational study, performed as part of the CONFER project. A call to all ECCO members was made to report concomitant CSy and 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 centers. 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). 6 patients had an underlying ulcerative colitis (UC), 16 Crohn`s disease (CD). 11 patients (50%) had active disease at CSy diagnosis. 16 patients were under IBD treatments at the time of CSy diagnosis, of whom 6 (27.3%) were on anti-TNF. 7 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 corticosteriods 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 phenomena.

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