Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is an extremely rare type of vasculitis characterized by inflammation within small blood vessels or tissues that may cause damage to the lungs, heart, kidneys, and other organs. Here, we present a rare case of EGPA with cardiac involvement that presented with acute heart failure.
Clinical findings: A 44-year-old woman with a history of bronchial asthma and sinusitis pre-sented with fever, shortness of breath, fatigue, unintentional weight loss, and polyarthritis. Physical examination revealed a fine basal crepitation and mononeuritis multiplex.
Diagnosis: The peripheral film revealed dimorphic features, an elevated eosinophil count, low hemoglobin (Hb), and abnormally high lactate dehydrogenase levels. The cardiac enzyme levels were elevated upon admission. Cardiac magnetic resonance imaging (CMR) revealed global hypokinesia and features suggestive of myocarditis. Echocardiography showed a low ejection fraction of 25%. Thus, the patient diagnosed with EPGA and myocarditis presented with acute heart failure.
Interventions: The patient was administered high-dose corticosteroids (intravenous bolus methylprednisolone 500 mg for three days, followed by 1 mg/kg of prednisolone) and cyclo-phosphamide 750 mg intravenously.
Outcome: After one months, the patient showed a marked improvement in clinical and labora-tory parameters. The ejection fraction improved to 30-40%, the eosinophil count returned to normal, and the hemolytic anemia resolved. The patient was sent home with mycophenolate mofetil 1 g twice a day as maintenance therapy.
Conclusion:
Patients with EGPA have a higher morbidity and mortality rate when they have cardiac in-volvement. The pathophysiological mechanism of cardiac involvement in EGPA warrants con-sideration of immunosuppressive therapy in addition to standard heart failure treatment.