Abstract
Granulomatosis with polyangiitis (GPA), formerly known as Wegener's disease, is a rare necrotizing and granulomatous systemic vasculitis of small vessels. Cardiac involvement in GPA is unusual and the heart is part of the so-called atypical locations of this vasculitis. This complication is often little-known and overlooked by clinicians despite its severity and high mortality. Symptomatic acute myocardial infarction is an exceptional presentation of cardiac involvement associated to GPA. We report an original observation of fatal cardiac damage revealing GPA in a 50-year-old Tunisian man with no pathological medical history. The diagnosis was made on post-mortem examination.
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