Presentation

A 61-year-old man presented with a 2-month history of tinnitus, progressive hearing loss, and a 2-week history of generalized joint pain, blurred vision, and redness in both eyes. He also had a high fever for the first 2 days and a positive PCR test for SARS-COV-2 RNA. On admission, he was afebrile, and the physical examination revealed bilateral conjunctival congestion (Fig. 1A). The right knee and interphalangeal joints were swollen and tender. A laboratory evaluation showed elevated CRP (19 mg/L) and ESR (40 mm/h). Autoantibodies (such as ANA, ENA, and ANCA) and pathogen examinations including treponema pallidum were negative. The audiometry indicated moderate to severe mixed deafness, and the internal otoscopy revealed left-sided secretory otitis media. Similarly, the MRI showed left-side otitis media and mastoiditis (Fig. 1B). Based on these data, we diagnosed Cogan syndrome (CS) after excluding syphilis, ANCA-associated vasculitis and other autoimmune diseases. With high-dose methylprednisolone (120 mg daily), his hearing and vision improved. After the tapering of glucocorticoid, he was treated with intravenous cyclophosphamide monthly, and the symptoms ameliorated significantly.

Fig. 1
figure 1

A Photograph of the patient’s eyes with bilateral conjunctival congestion. B The MRI (T2-weighted) shows left-side otitis media and mastoiditis

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Discussion

CS is a rare autoimmune systemic vasculitis of unknown etiology, characterized by ocular inflammation and vestibuloauditory dysfunction [1]. The pathogenic mechanism of COVID-19-associated vasculitis is not fully understood. It is thought that SARS-COV-2 may increase angiotensin II, while the type I interferon response to the SARS-COV-2 may decrease nitric oxide production [2, 3]. Furthermore, immune dysregulation and autoantibody reactions can also be involved in the pathogenesis [4]. These changes can result in vasoconstriction and endothelial damage, and ultimately lead to vasculitis [5].

In summary, our report deepens the understanding of the link between COVID-19 and systemic vasculitis, but the phathogenesis of COVID-19-related vasculitis requires further investigation. Vasculitis triggered by COVID-19 has a reported worldwide prevalence, of which timely diagnosis and treatment can significantly improve the prognosis.