Cogan Syndrome with Aortic Regurgitation and Multiple Vasculopathy.

A 57-year-old woman with a history of scleritis was referred to the cardiology clinic due to palpitation. One year earlier, she had been diagnosed with Cogan syndrome (CS). Computed tomography revealed aortic wall thickening (Picture 1, left arrowheads), which improved with immunosuppressive therapy, including prednisolone, methotrexate, and tocilizumab (Picture 1, right arrows). The transthoracic echocardiography revealed severe aortic regurgitation (Picture 2, arrow). The peripheral angiography showed severe right brachial artery stenosis (Picture 3, left, arrow), and coronary angiography showed moderate stenoses of the left and right coronary arteries (Picture 3, right, arrowhead). The patient underwent aortic valve replacement. The histopathologic microphotograph derived from the resected aortic valves exhibited prominent myxomatous degeneration (Picture 4), although this finding was not specific to CS. CS is a rare and chronic vasculitis, characterized by keratitis, scleritis, arteritis, and valvulitis (1). Our observations suggest that cardiovascular diseases should be meticulously evaluated as possible comorbidities of CS, as a delayed diagnosis can be fatal.

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The authors state that they have no Conflict of Interest (COI).