Background/Purpose: Cogan syndrome (CS) is an extremely rare autoimmune systemic vasculitis characterized by intraocular inflammation and vestibulo-auditory dysfunction. The global prevalence of this disease is currently not known. There are fewer than 250 reported cases reported with no noted gender predilections. The actual number of individuals with Cogan’s syndrome could be higher than reported as many cases may be incorrectly diagnosed as idiopathic hearing loss/deafness, autoimmune inner ear disease and idiopathic recurring keratitis. We sought to investigate the global prevalence and clinical outcomes among patients with typical CS in a global research database.

Methods: This study was conducted using Trinetx, a global federated health research network providing access to electronic medical records across large healthcare organizations (HCOs). Using ICD-10 codes, patients diagnosed with Cogan Syndrome over the past 20 years were identified. We assessed the prevalence as well as the outcomes of blindness, hearing loss, aortic insufficiency and heart failure within 5 years of diagnosis.

Results: A total of 126,851,661 patients were queried on June 9, 2024. The prevalence of CS was found to be 0.00015% (1,930). The mean age of diagnosis was 50, females were more commonly affected, and more than half of patients were Caucasian. The risk of hearing loss was 19.6%, 13% had vertigo, 5% developed aortitis and aortic valve insufficiency, and 5.8% developed heart failure. Only 2% of patients were diagnosed with blindness within 5 years after diagnosis.

Conclusion: CS is an uncommon disease with a prevalence of 0.00015%. Unlike prior case reports, this data supports a female predominance in CS. The major disease-related morbidity is hearing loss followed by vertigo and infrequently aortitis and blindness.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/global-prevalence-and-clinical-outcomes-of-cogan-syndrome/