IntroductionThis case report describes the delay in diagnosis and potentially life-threatening complications of a patient with large vessel vasculitis.The initial diagnosis was of vascular Ehlers-Danlos Syndrome, based on family history and imaging. However, recurrent episodes of chest pain and a rapidly expanding aortic aneurysm prompted further diagnostics which led to the diagnosis of giant cell arteritis with large vessel vasculitis. The patient required thoracic surgery due to expansion of the aortic aneurysm. This case demonstrates the risk of delaying the diagnosis of large vessel vasculitis and the complexities of immunosuppression in the context of major surgery.
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