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  • Title: Atypical Cogan's syndrome: a case report and summary of current treatment options.
    Author: Jančatová D, Zeleník K, Komínek P, Matoušek P.
    Journal: Int J Pediatr Otorhinolaryngol; 2015 Mar; 79(3):428-31. PubMed ID: 25600284.
    Abstract:
    Cogan's syndrome is a rare chronic vasculitis, characterized in its typical form by progressive sensorineural bilateral hearing loss, vestibular symptoms and non-syphilitic interstitial keratitis. Only a few cases have been reported in children, most of whom have been diagnosed with the typical form. Early diagnosis and treatment are crucial to ensure a favorable prognosis. Systemic treatment usually begins with high dosage corticosteroids. In case the initial treatment fails, other immunosuppressive drugs are used (cyclophosphamide, methotrexate, cyclosporine A and azathioprine). Additional treatment possibilities, such as plasmapheresis, TNF-alpha blockers (etanercept and infliximab), rituximab, tocilizumab and mycophenolate mofetil have been described over the past few years.
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