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CASE REPORT article

Front. Cardiovasc. Med.
Sec. General Cardiovascular Medicine
Volume 11 - 2024 | doi: 10.3389/fcvm.2024.1490735
This article is part of the Research Topic Case Reports in General Cardiovascular Medicine: 2024 View all 3 articles

Eosinophilic granulomatosis with polyangiitis; a Distinctive Presentation with Myocarditis and Autoimmune Haemolytic Anaemia: Case Report

Provisionally accepted
Doaa Ayish Doaa Ayish 1Fatma Ayish Fatma Ayish 1Asayel Khamjan Asayel Khamjan 2Amal H Mohamed Amal H Mohamed 1Nagla Abdalgani Nagla Abdalgani 1Osama Albasheer Osama Albasheer 1*
  • 1 Faculty of Medicine, Jazan University, Jizan, Saudi Arabia
  • 2 Ministry of Health (Saudi Arabia), Riyadh, Riyadh, Saudi Arabia

The final, formatted version of the article will be published soon.

    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.A 44-year-old woman with a history of bronchial asthma and sinusitis presented with fever, shortness of breath, fatigue, unintentional weight loss, and polyarthritis. Physical examination revealed bilateral basal crepitation and mononeuritis multiplex.The peripheral blood smear revealed red blood cells of different sizes and shapes (dimorphic features), more eosinophils, low hemoglobin, and higher lactate dehydrogenase (LDH) levels. 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.The patient was administered high-dose corticosteroids (intravenous bolus methylprednisolone 500 mg for three days, followed by 1 mg/kg of prednisolone) and cyclophosphamide 750 mg intravenously.Outcome: After one months, the patient showed a marked improvement in clinical and laboratory parameters. The ejection fraction improved to 30-40%, the eosinophil count returned to normal, and the haemolytic anaemia resolved. The patient was sent home and shifted to mycophenolate mofetil 1 g twice a day as maintenance therapy.Patients with EGPA have a higher morbidity and mortality rate when they have cardiac involvement. The pathophysiological mechanism of cardiac involvement in EGPA warrants consideration of immunosuppressive therapy in addition to standard heart failure treatment.

    Keywords: Eosinophilia, Myocarditis, Vasculitis, Autoimmune haemolytic anaemia, heart failure Churg-Strauss syndrome CSS Anti-neutrophil cytoplasmic antibody ANCA Cardiac magnetic resonance CMR Autoimmune hemolytic anemia AIHA

    Received: 27 Sep 2024; Accepted: 19 Dec 2024.

    Copyright: © 2024 Ayish, Ayish, Khamjan, Mohamed, Abdalgani and Albasheer. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence: Osama Albasheer, Faculty of Medicine, Jazan University, Jizan, Saudi Arabia

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