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Cogan’s syndrome is a chronic inflammatory disorder that most commonly affects young adults. Clinical hallmarks are interstitial keratitis and vestibule-auditory dysfunction in combination perhaps with systemic vasculitis, and aortitis. The predominant ocular manifestation of Cogan’s syndrome is interstitial keratitis (which typically causes eye redness, pain, photophobia, and blurred vision). Inner ear manifestations are included Meniere-like attacks consisting of vertigo, ataxia, nausea, vomiting, tinnitus, and hearing loss and vestibular dysfunction which can cause oscillopsia. Systemic vasculitis with large- or medium- to small-sized vessel vasculitis or aortitis is also of great significance. Systemic manifestations have been reported in < 5% of patients. Therapeutic options for the treatment of Cogan’s syndrome include the use of corticosteroids (topical agents or systemic administration) and in resistant cases, immunosuppressive therapy (cyclophosphamide, cyclosporine, methotrexate, leflunomide, mycophenolate mofetil). In cases with poor response to the aforementioned treatment, the administration of Anti-TNFα therapy (Infliximab) or Rituximab (a monoclonal antibody inducing depletion of B lymphocytes) offered successful and rapid improvement of the manifestations of the eye, the inner ear and the cardiovascular system.