To review the clinical features of Cogan syndrome, a rare vasculitis characterized by systemic, ocular, and audiovestibular symptoms.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.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\%).Audiovestibular and ocular involvement have a major impact on prognosis in children with Cogan syndrome.

Clinical features and outcome of Cogan syndrome / I. Pagnini;M. E. Zannin;F. Vittadello;M. Sari;G. Simonini;R. Cimaz;F. Zulian. - In: THE JOURNAL OF PEDIATRICS. - ISSN 0022-3476. - STAMPA. - 160:(2012), pp. 303-307.e1. [10.1016/j.jpeds.2011.07.051]

Clinical features and outcome of Cogan syndrome.

PAGNINI, ILARIA;SIMONINI, GABRIELE;CIMAZ, ROLANDO;
2012

Abstract

To review the clinical features of Cogan syndrome, a rare vasculitis characterized by systemic, ocular, and audiovestibular symptoms.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.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\%).Audiovestibular and ocular involvement have a major impact on prognosis in children with Cogan syndrome.
2012
160
303
307.e1
I. Pagnini;M. E. Zannin;F. Vittadello;M. Sari;G. Simonini;R. Cimaz;F. Zulian
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Utilizza questo identificatore per citare o creare un link a questa risorsa: https://hdl.handle.net/2158/973829
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