Síndrome de Cogan: análisis descriptivo y experiencia clínica de 7 casos diagnosticados y tratados en 2 hospitales de tercer nivel / Cogan syndrome: Descriptive analysis and clinical experience of 7 cases diagnosed and treated in two third level hospitals

Objetivo: El síndrome de Cogan (SC) es una enfermedad inflamatoria clasificada como vasculitis de vaso variable. Se trata de una enfermedad rara con escasas series publicadas, por lo que revisamos nuestra experiencia en 2 centros en los últimos 10 años. Material y métodos: Descripción de 7 casos diagnosticados de SC, atendiendo a los criterios de clasificación (típico o atípico), sus manifestaciones clínicas, tratamientos utilizados y sus complicaciones. Se realizó un análisis comparativo con las series y casos descritos en la literatura. Resultados: Se incluyeron 7 casos, 3 varones y 4 mujeres, con una edad media al diagnóstico de 43 años, y un tiempo de evolución medio de 47 meses. Cinco pacientes cumplían las características típicas según los criterios clásicos de 1980, siendo el resto casos atípicos, uno por ausencia de queratitis intersticial y otro por un periodo entre la aparición de clínica ocular y auditivo-vestibular mayor de 2 años. Todos recibieron inmunosupresores, siendo el más utilizado el metotrexato, seguido de la azatioprina. En 5 casos se utilizaron fármacos biológicos: infliximab en 4 ocasiones y en 2 tocilizumab. Un paciente falleció por endocarditis bacteriana y shock séptico. Conclusión: Las características de la serie presentada son similares a las publicadas hasta ahora, con diferencias clínicas fundamentalmente en la afectación de grandes vasos. Ante la escasa casuística, parece necesario la creación de registros multicéntricos para mejorar la evidencia en cuanto al manejo de pacientes con SC.(AU)

Objective: Cogan's syndrome (CS) is an inflammatory disease classified as variable vessel vasculitis. It is a rare disease with few published series, and therefore we reviewed our experience in the last ten years in two centres. Materials and methods: Description of 7 diagnosed cases of CS, according to the classification criteria (typical or atypical), their clinical manifestations, treatments used and their complications. A comparative analysis was performed with the series and cases described in the literature. Results: Seven cases were included, three men and four women, with a mean age at diagnosis of 43 years, and an average disease duration of 47 months. Five patients met the typical characteristics according to the 1980 classical criteria, the rest being atypical cases, one due to the absence of interstitial keratitis and another due to a period between the onset of ocular and auditory-vestibular clinical symptoms greater than two years. All received immunosuppressants, methotrexate being the most commonly used, followed by azathioprine. In 5 cases, biological drugs were used, infliximab in 4 times and 2 tocilizumab. One patient died from bacterial endocarditis and septic shock. Conclusion: The characteristics of the series presented are like those published to date, with clinical differences mainly in the involvement of large vessels. Given the low frequency, it seems necessary to create multicentre records to improve the evidence regarding the management of patients with CS.(AU)

Cogan syndrome: Descriptive analysis and clinical experience of 7 cases diagnosed and treated in two third level hospitals. / Síndrome de Cogan: análisis descriptivo y experiencia clínica de 7 casos diagnosticados y tratados en 2 hospitales de tercer nivel.

OBJECTIVE: Cogan's syndrome (CS) is an inflammatory disease classified as variable vessel vasculitis. It is a rare disease with few published series, and therefore we reviewed our experience in the last ten years in two centres. MATERIALS AND METHODS: Description of 7 diagnosed cases of CS, according to the classification criteria (typical or atypical), their clinical manifestations, treatments used and their complications. A comparative analysis was performed with the series and cases described in the literature. RESULTS: Seven cases were included, three men and four women, with a mean age at diagnosis of 43 years, and an average disease duration of 47 months. Five patients met the typical characteristics according to the 1980 classical criteria, the rest being atypical cases, one due to the absence of interstitial keratitis and another due to a period between the onset of ocular and auditory-vestibular clinical symptoms greater than two years. All received immunosuppressants, methotrexate being the most commonly used, followed by azathioprine. In 5 cases, biological drugs were used, infliximab in 4 times and 2 tocilizumab. One patient died from bacterial endocarditis and septic shock. CONCLUSION: The characteristics of the series presented are like those published to date, with clinical differences mainly in the involvement of large vessels. Given the low frequency, it seems necessary to create multicentre records to improve the evidence regarding the management of patients with CS.