[Syncope, pain in the large joints, and painful swelling of the right eye in a 51-year-old patient : Pitfalls in the diagnosis and treatment of a rare disease]

In the present case we report on a 51-year-old patient diagnosed with Cogan syndrome. This vasculitis of variable vessel size is a rare disease that poses a major challenge for the correct diagnostics and therapy. In the classic setting, it comprises a triad of non-syphilitic interstitial keratitis as well as hearing loss with vestibular dysfunction. A vascultis-related aortitis, an uncertain, more likely degenerative structure in combination with strongly elevated inflammation parameters was misinterpreted as infective endocarditis for a long time and treated with anti-infective medications. After diagnosis the patient recovered following treatment with high-dose steroids and in the further course cyclophosphamide and tumor necrosis factor‑α blockers.

Keywords: Aortitis; Cogan syndrome; Endocarditis; Nephrotic syndrome; Vasculitis.