Summary

Granulomatosis with polyangiitis (GPA) is a systemic vasculitis characterised by necrotising inflammatory changes in small-sized and medium-sized vessels and granuloma formation. It most commonly involves the kidneys and respiratory tract, but it can present with widespread manifestations involving any organ system. Rarely, it causes coronary vasculitis which can precipitate a severe cardiomyopathy. Here, we report a patient who presented in cardiogenic shock requiring vasopressors and was found to have extensive myocardial ischaemia secondary to coronary vasculitis. Further investigation led to a diagnosis of GPA, and he responded to treatment with corticosteroids, cyclophosphamide and plasmapheresis.