Cogan's syndrome: present and future directions.

Hdl Handle:
http://hdl.handle.net/10147/200965
Title:
Cogan's syndrome: present and future directions.
Authors:
Murphy, Grainne; Sullivan, Miriam O; Shanahan, Fergus; Harney, Sinead; Molloy, Michael
Affiliation:
Department of Rheumatology, Cork University Hospital, Wilton, Cork, Ireland. grainne927@yahoo.com
Citation:
Cogan's syndrome: present and future directions. 2009, 29 (10):1117-21 Rheumatol. Int.
Journal:
Rheumatology international
Issue Date:
Aug-2009
URI:
http://hdl.handle.net/10147/200965
DOI:
10.1007/s00296-009-0945-0
PubMed ID:
19471934
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.
Item Type:
Article
Language:
en
Description:
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.
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
ISSN:
1437-160X

Full metadata record

DC FieldValue Language
dc.contributor.authorMurphy, Grainneen
dc.contributor.authorSullivan, Miriam Oen
dc.contributor.authorShanahan, Fergusen
dc.contributor.authorHarney, Sineaden
dc.contributor.authorMolloy, Michaelen
dc.date.accessioned2012-01-09T16:17:07Z-
dc.date.available2012-01-09T16:17:07Z-
dc.date.issued2009-08-
dc.identifier.citationCogan's syndrome: present and future directions. 2009, 29 (10):1117-21 Rheumatol. Int.en
dc.identifier.issn1437-160X-
dc.identifier.pmid19471934-
dc.identifier.doi10.1007/s00296-009-0945-0-
dc.identifier.urihttp://hdl.handle.net/10147/200965-
dc.descriptionCogan'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.en
dc.description.abstractCogan'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.-
dc.language.isoenen
dc.subject.meshAdrenal Cortex Hormones-
dc.subject.meshAdult-
dc.subject.meshAzathioprine-
dc.subject.meshDose-Response Relationship, Drug-
dc.subject.meshFatal Outcome-
dc.subject.meshFemale-
dc.subject.meshHearing Loss, Sensorineural-
dc.subject.meshHumans-
dc.subject.meshKeratitis-
dc.subject.meshPractice Guidelines as Topic-
dc.subject.meshPrognosis-
dc.subject.meshRandomized Controlled Trials as Topic-
dc.subject.meshSyndrome-
dc.subject.meshUveitis-
dc.subject.meshVascular Diseases-
dc.titleCogan's syndrome: present and future directions.en
dc.typeArticleen
dc.contributor.departmentDepartment of Rheumatology, Cork University Hospital, Wilton, Cork, Ireland. grainne927@yahoo.comen
dc.identifier.journalRheumatology internationalen
dc.description.provinceMunster-

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