Clinical Features and Outcome of Cogan Syndrome - 10/01/12
, Francesco Zulian, MD 2, ⁎ 
Reprint requests: Francesco Zulian, MD, Dipartimento di Pediatria, Università di Padova, Via Giustiniani 3, 35128 Padova, Italy.Abstract |
Objective |
To review the clinical features of Cogan syndrome, a rare vasculitis characterized by systemic, ocular, and audiovestibular symptoms.
Study design |
Clinical records of patients with Cogan syndrome followed at 2 pediatric rheumatology institutions and those from a database search were reviewed. Data included clinical features at onset and during the disease course, treatments, and outcomes.
Results |
Twenty-three children with Cogan syndrome (15 males; mean age, 11.4 years [range, 4-18 years]) were included in the analysis. Eleven patients (47.8%) exhibited systemic features at disease onset, including fever, arthralgias-arthritis or myalgias, headache, and weight loss. Twenty-one patients (91.3%) had ocular symptoms, due mainly to interstitial keratitis, uveitis, or conjunctivitis/episcleritis. Vestibular symptoms (39.1%) included vertigo, vomiting, and dizziness. Auditory involvement (65.2%) consisted of sensorineural hearing loss, tinnitus, and deafness. Four patients had cardiac valve involvement, and 3 had skin manifestations. After a median 2 years of follow-up, 30.4% of the patients were in clinical remission, but all others had irreversible complications (deafness, 21.7%; sensorineural hearing loss, 13.0%; vestibular dysfunction, 4.3%; ocular complications, 13.0%; cardiac valve damage, 17.4%).
Conclusion |
Audiovestibular and ocular involvement have a major impact on prognosis in children with Cogan syndrome.
Le texte complet de cet article est disponible en PDF.Mots-clés : ENT, MRI, NSAID, SNHL
Plan
| The authors declare no conflicts of interest. |
Vol 160 - N° 2
P. 303 - février 2012 Retour au numéro
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