title: Clinical features and outcome of Cogan syndrome.
abstract: OBJECTIVE
  To review the clinical features of Cogan syndrome, a rare vasculitis characterized by systemic, ocular, and audiovestibular symptoms.
  
  
  METHODS
  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%).
  
  
  CONCLUSIONS
  Audiovestibular and ocular involvement have a major impact on prognosis in children with Cogan syndrome.
authors.affiliation: Department of Pediatrics, University of Florence, Florence, Italy.
authors.forename: Ilaria
authors.initials: I
authors.lastname: Pagnini
authors.forename: Maria Elisabetta
authors.initials: ME
authors.lastname: Zannin
authors.forename: Fabio
authors.initials: F
authors.lastname: Vittadello
authors.forename: Marianna
authors.initials: M
authors.lastname: Sari
authors.forename: Gabriele
authors.initials: G
authors.lastname: Simonini
authors.forename: Rolando
authors.initials: R
authors.lastname: Cimaz
authors.forename: Francesco
authors.initials: F
authors.lastname: Zulian
mesh_terms: D000293:Adolescent; D055952:Cogan Syndrome / Q000150:complications* / Q000503:physiopathology*; D003638:Deafness / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007634:Keratitis / Q000503:physiopathology; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D014605:Uveitis / Q000503:physiopathology; D014657:Vasculitis / Q000503:physiopathology; D014717:Vertigo / Q000503:physiopathology