Síndrome de Cogan asociado a vasculitis sistémica con evolución tórpida

Introduction

Cogan's syndrome is a rare autoimmune disease characterized by ocular and auditory manifestations. The key symptoms are interstitial keratitis and sudden hearing loss. Many patients also present systemic compromise, vasculitis being a rare manifestation of the disease.

Case report

62-year-old female patient who consulted for transient brachial dysesthesias and after a complete evaluation the diagnosis of ischemic cerebrovascular accident (CVA) of undetermined cause was made. Later, she presented a new cerebrovascular event, for which she was studied again, without pathological findings. In the following two months, the presence of systemic symptoms (headache, weight loss), bilateral hearing loss, episodes of vertigo and a new stroke were added to the clinical picture. Due to suspicion of systemic vasculitis, positron emission tomography was performed with evidence of aortic and femoral vasculitis. The condition was interpreted as Cogan's syndrome and immunosuppressive treatment was started with daily oral meprednisone and monthly cyclophosphamide. Despite treatment, the patient evolved with a new extensive stroke associated with thrombosis of both carotid arteries. Plasmapheresis was performed, with no response since the patient died 4 months after the clinical picture began.

Conclusion

The presentation of systemic manifestations of Cogan's syndrome prior to visual and auditory involvement represent a rare onset of the disease. Likewise, stroke as an initial manifestation is extremely rare, so it should be suspected in cases of repetitive events in patients with no other apparent causes.