title: Cogan's Syndrome in Patients With Inflammatory Bowel Disease--A Case Series.
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.
  
  
  CONCLUSIONS
  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.affiliation: Division of Gastroenterology and Hepatology, University Hospital Zurich, Zurich, Switzerland Division of Gastroenterology and Hepatology, Triemli Hospital Zurich, Zurich, Switzerland.
authors.forename: Stephan R
authors.initials: SR
authors.lastname: Vavricka
authors.affiliation: Division of Gastroenterology and Hepatology, University Hospital Zurich, Zurich, Switzerland.
authors.forename: Thomas
authors.initials: T
authors.lastname: Greuter
authors.affiliation: Division of Gastroenterology and Hepatology, University Hospital Zurich, Zurich, Switzerland.
authors.forename: Michael
authors.initials: M
authors.lastname: Scharl
authors.affiliation: Department of Gastroenterology, Evangelismos Hospital, Athens, Greece.
authors.forename: Gerassimos
authors.initials: G
authors.lastname: Mantzaris
authors.affiliation: Digestive Diseases Institute, Shaare Zedek Medical Center, Hebrew University Medical School, Jerusalem, Israel.
authors.forename: Ariella B
authors.initials: AB
authors.lastname: Shitrit
authors.affiliation: Department of Clinical Endoscopy, Institute of Rural Health, Lublin, Poland.
authors.forename: Rafal
authors.initials: R
authors.lastname: Filip
authors.affiliation: Department of Gastroenterology, Venizeleio General Hospital, Heraklion, Crete, Greece.
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Karmiris
authors.affiliation: II. Medizinische Klinik und Poliklinik, Klinikum rechts der Isar, Technical University of Munich, Munich, Germany.
authors.forename: Christoph K
authors.initials: CK
authors.lastname: Thoeringer
authors.affiliation: Department of Gastroenterology and Hepatology, Medical University of Silesia, Katowice, Poland.
authors.forename: Hubert
authors.initials: H
authors.lastname: Boldys
authors.affiliation: Department of Pediatrics, Hvidovre University Hospital, Hvidovre, Denmark.
authors.forename: Anne V
authors.initials: AV
authors.lastname: Wewer
authors.affiliation: 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.
authors.forename: Henit
authors.initials: H
authors.lastname: Yanai
authors.affiliation: Gastroenterology Section, Porto Alegre Clinical Hospital, Federal University of Rio Grande do Sul, Porto Alegre, Brasil.
authors.forename: Cristina
authors.initials: C
authors.lastname: Flores
authors.affiliation: Department of Gastroenterology, University Hospital Jena, Jena, Germany.
authors.forename: Carsten
authors.initials: C
authors.lastname: Schmidt
authors.affiliation: Department of Gastroenterology and Liver Diseases, Tel Aviv Medical Center, Tel Aviv, Israel.
authors.forename: Revital
authors.initials: R
authors.lastname: Kariv
authors.affiliation: Division of Gastroenterology and Hepatology, University Hospital Zurich, Zurich, Switzerland.
authors.forename: Gerhard
authors.initials: G
authors.lastname: Rogler
authors.affiliation: Department of Hepatogastroenterology, CHU Dinant Godinne, UCL Namur, Yvoir, Belgium.
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Rahier
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D055952:Cogan Syndrome / Q000150:complications* / Q000175:diagnosis*; D003093:Colitis, Ulcerative / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D003424:Crohn Disease / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D005260:Female; D005765:Gastrointestinal Agents / Q000627:therapeutic use; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D005765:Gastrointestinal Agents; D007155:Immunologic Factors
keywords: Cogan’s syndrome; Crohn’s disease; inflammatory bowel disease; ulcerative colitis