RESUMO
Cogan's syndrome is a rare autoimmune disease that usually affects young Caucasian adults and is classically defined as the combination of nonsyphilitic interstitial keratitis and audiovestibular symptoms resembling Meniere's disease, both of them developed in an interval of less than two years. Nevertheless, cases with atypical ophthalmologic and audiovestibular features, with systemic manifestations or affecting children and older patients have also been reported, expanding the clinical spectrum of Cogan's syndrome. Herein, we present the case of a late-onset Cogan's syndrome associated with a large-vessel vasculitis
El síndrome de Cogan es una enfermedad autoinmune rara, que afecta frecuentemente a pacientes jóvenes de raza caucásica y que se define clásicamente por la combinación de queratitis intersticial no sifilítica y síntomas audiovestibulares similares a una enfermedad de Ménière, que se desarrollan en un intervalo de menos de 2 años. Sin embargo, se han descrito casos con manifestaciones oftalmológicas o audiovestibulares atípicas, con síntomas sistémicos o que afectan a niños o pacientes ancianos, expandiendo de este modo el espectro clínico del síndrome de Cogan. Presentamos aquí el caso de un síndrome de Cogan de inicio tardío asociado con una vasculitis de gran vaso
Assuntos
Humanos , Feminino , Idoso de 80 Anos ou mais , Aortite/complicações , Síndrome de Cogan/complicações , Idade de Início , Aortite/diagnóstico por imagem , Síndrome de Cogan/diagnóstico , Tomografia por Emissão de Pósitrons , Tomografia Computadorizada por Raios X , Artéria Subclávia/diagnóstico por imagemRESUMO
Cogan's syndrome is a rare autoimmune disease that usually affects young Caucasian adults and is classically defined as the combination of nonsyphilitic interstitial keratitis and audiovestibular symptoms resembling Meniere's disease, both of them developed in an interval of less than two years. Nevertheless, cases with atypical ophthalmologic and audiovestibular features, with systemic manifestations or affecting children and older patients have also been reported, expanding the clinical spectrum of Cogan's syndrome. Herein, we present the case of a late-onset Cogan's syndrome associated with a large-vessel vasculitis.
