Cogan's syndrome: present and future directions.
Murphy, Grainne Sullivan, Miriam O Shanahan, Fergus Harney, Sinead Molloy, Michael
Murphy, Grainne
Sullivan, Miriam O
Shanahan, Fergus
Harney, Sinead
Molloy, Michael
Advisors
Editors
Other Contributors
Date
2009-08
Date Submitted
Keywords
Other Subjects
Subject Mesh
Adrenal Cortex Hormones
Adult
Azathioprine
Dose-Response Relationship, Drug
Fatal Outcome
Female
Hearing Loss, Sensorineural
Humans
Keratitis
Practice Guidelines as Topic
Prognosis
Randomized Controlled Trials as Topic
Syndrome
Uveitis
Vascular Diseases
Adult
Azathioprine
Dose-Response Relationship, Drug
Fatal Outcome
Female
Hearing Loss, Sensorineural
Humans
Keratitis
Practice Guidelines as Topic
Prognosis
Randomized Controlled Trials as Topic
Syndrome
Uveitis
Vascular Diseases
Planned Date
Start Date
Collaborators
Principal Investigators
Alternative Titles
Publisher
Abstract
Cogan's syndrome, typified by the combination of interstitial keratitis and immune-mediated sensorineural hearing loss, is a rare condition, and commonly associated with a diagnostic delay. Using a standard search protocol, we review the literature to date, focusing on a number of key areas pertaining to diagnosis, presentation and treatment. Using a case illustration of atypical disease which led to fulminant aortic regurgitation, we highlight the need for continued and collaborative research in order to identify negative prognostic factors and thus tailor therapeutic regimens. Atypical Cogan's syndrome is more commonly associated with systemic manifestations than typical disease, and may be refractory to immunosuppressive treatment. We discuss the application of laboratory (e.g antibodies targeting inner ear antigens) and radiological (PET-CT) aids to disease confirmation and detection of sub-clinical vascular inflammation. As illustrated by the included case description, some patients remain refractory to intense immunosuppression and delineation of adverse prognostic factors which may direct treatment, perhaps including the use of PET-CT, will contribute in the future to improving patient outcomes.
Language
en
ISSN
1437-160X
eISSN
ISBN
DOI
10.1007/s00296-009-0945-0
PMID
19471934