A fiery heart: case report of perimyocarditis in a patient with eosinophilic granulomatosis with polyangiitis

Abstract

Background Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare disease caused by small- to medium-sized vessel vasculitis which can also impact the heart. Because of its rarity and diverse clinical manifestations, diagnosis can be challenging. Here, we present a unique case of EGPA causing perimyocarditis in a young female patient. Case summary A 37-year-old woman with hypertension, asthma, and sickle cell trait presented with palpitations, dyspnoea, and sharp chest pain. White blood cell was elevated to 16 300/μL with peripheral eosinophilia at 5216/μL. Electrocardiogram revealed sinus tachycardia with frequent non-sustained ventricular tachycardia. Echocardiogram showed an ejection fraction of 20–25% with severe diffuse hypokinesis and dilated cardiac chambers. Coronary angiogram was normal. Cardiac magnetic resonance imaging revealed focal subendocardial late gadolinium enhancement (LGE) of the septum and subepicardial LGE of the basal anterolateral wall of the left ventricle. Further work-up showed elevated Immunoglobulin E level, left antrochoanal polyp, and ground glass opacities in the left upper lobe. Endomyocardial biopsy showed interstitial infiltrates of eosinophils with sporadic necrosis, confirming the diagnosis of EGPA perimyocarditis. The patient was treated with prednisone, colchicine, and guideline-directed medical therapy. Discussion This case report describes an unusual cause of perimyocarditis. Keeping a broad differential is important as diagnosis is challenging, and cardiac involvement in EGPA is associated with higher morbidity and mortality. Recognizing the typical manifestations of EGPA, implementing multidisciplinary approach, and promptly initiating appropriate treatment are crucial for the optimal management of EGPA perimyocarditis.