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

Stephan R Vavricka; Thomas Greuter; Michael Scharl; Gerassimos Mantzaris; Ariella B Shitrit; Rafal Filip; Konstantinos Karmiris; Christoph K Thoeringer; Hubert Boldys; Anne Vibeke Wewer; Henit Yanai; Cristina Flores; Carsten Schmidt; Revital Kariv; Gerhard Rogler; Jean-François Rahier; ECCO CONFER investigators

doi:10.1093/ecco-jcc/jjv128

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

Stephan R Vavricka, Thomas Greuter, Michael Scharl, Gerassimos Mantzaris, Ariella B Shitrit, Rafal Filip, Konstantinos Karmiris, Christoph K Thoeringer, Hubert Boldys, Anne Vibeke Wewer, Henit Yanai, Cristina Flores, Carsten Schmidt, Revital Kariv, Gerhard Rogler, Jean-François Rahier, ECCO CONFER investigators

Department of Clinical Medicine

13 Citations (Scopus)

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.

Original language	English
Journal	Journal of Crohn's & colitis
Volume	9
Issue number	10
Pages (from-to)	886-90
Number of pages	5
ISSN	1873-9946
DOIs	https://doi.org/10.1093/ecco-jcc/jjv128
Publication status	Published - 1 Oct 2015

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Vavricka, S. R., Greuter, T., Scharl, M., Mantzaris, G., Shitrit, A. B., Filip, R., Karmiris, K., Thoeringer, C. K., Boldys, H., Wewer, A. V., Yanai, H., Flores, C., Schmidt, C., Kariv, R., Rogler, G., Rahier, J-F., & ECCO CONFER investigators (2015). Cogan's Syndrome in Patients With Inflammatory Bowel Disease--A Case Series. Journal of Crohn's & colitis, 9(10), 886-90. https://doi.org/10.1093/ecco-jcc/jjv128

Vavricka, SR, Greuter, T, Scharl, M, Mantzaris, G, Shitrit, AB, Filip, R, Karmiris, K, Thoeringer, CK, Boldys, H, Wewer, AV, Yanai, H, Flores, C, Schmidt, C, Kariv, R, Rogler, G, Rahier, J-F & ECCO CONFER investigators 2015, 'Cogan's Syndrome in Patients With Inflammatory Bowel Disease--A Case Series', Journal of Crohn's & colitis, vol. 9, no. 10, pp. 886-90. https://doi.org/10.1093/ecco-jcc/jjv128

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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.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.",

author = "Vavricka, {Stephan R} and Thomas Greuter and Michael Scharl and Gerassimos Mantzaris and Shitrit, {Ariella B} and Rafal Filip and Konstantinos Karmiris and Thoeringer, {Christoph K} and Hubert Boldys and Wewer, {Anne Vibeke} and Henit Yanai and Cristina Flores and Carsten Schmidt and Revital Kariv and Gerhard Rogler and Jean-Fran{\c c}ois Rahier and {ECCO CONFER investigators}",

note = "Copyright {\textcopyright} 2015 European Crohn{\textquoteright}s and Colitis Organisation (ECCO). Published by Oxford University Press. All rights reserved. For permissions, please email: [email protected].",

year = "2015",

month = oct,

day = "1",

doi = "10.1093/ecco-jcc/jjv128",

language = "English",

volume = "9",

pages = "886--90",

journal = "Journal of Crohn's and Colitis",

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TY - JOUR

T1 - Cogan's Syndrome in Patients With Inflammatory Bowel Disease--A Case Series

AU - Vavricka, Stephan R

AU - Greuter, Thomas

AU - Scharl, Michael

AU - Mantzaris, Gerassimos

AU - Shitrit, Ariella B

AU - Filip, Rafal

AU - Karmiris, Konstantinos

AU - Thoeringer, Christoph K

AU - Boldys, Hubert

AU - Wewer, Anne Vibeke

AU - Yanai, Henit

AU - Flores, Cristina

AU - Schmidt, Carsten

AU - Kariv, Revital

AU - Rogler, Gerhard

AU - Rahier, Jean-François

AU - ECCO CONFER investigators

PY - 2015/10/1

Y1 - 2015/10/1

N2 - 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.

AB - 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.

U2 - 10.1093/ecco-jcc/jjv128

DO - 10.1093/ecco-jcc/jjv128

M3 - Journal article

C2 - 26188351

SN - 1873-9946

VL - 9

SP - 886

EP - 890

JO - Journal of Crohn's and Colitis

JF - Journal of Crohn's and Colitis

IS - 10

ER -

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

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