Fatal Cardiac Involvement Revealing Granulomatosis with Polyangiitis

Salem Bouomrani^{1, 2,} , Karama Regaïeg³, Nesrine Belgacem¹, Mouna Guermazi^{1, 2}, Sami Krimi³ and Abdelfatteh Zakhama⁴

¹Department of Internal Medicine, Military Hospital of Gabes, Gabes 6000, Tunisia

²Sfax Faculty of Medicine, University of Sfax, Sfax 3029, Tunisia

³Department of Forensic Medicine, Gabes Regional Hospital, Gabes 6000, Tunisia

⁴Department of Pathology, Fattouma Bourguiba Hospital, Monastir 5000, Tunisia

Cite this paper:
Salem Bouomrani, Karama Regaïeg, Nesrine Belgacem, Mouna Guermazi, Sami Krimi and Abdelfatteh Zakhama. Fatal Cardiac Involvement Revealing Granulomatosis with Polyangiitis. American Journal of Medical Case Reports. 2019; 7(5):90-93. doi: 10.12691/ajmcr-7-5-4

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.

Keywords:
granulomatosis with polyangiitis Wegener’s disease cardiac involvement myocardial infarction sudden death vasculitis

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