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Case Report

Pancarditis as the Clinical Presentation of Eosinophilic Granulomatosis with Polyangiitis: A Multimodality Approach to Diagnosis

1
Inherited and Rare Cardiovascular Disease Unit, AORN dei Colli, Monaldi Hospital, 80131 Naples, Italy
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Division of Cardiology, Villa dei Fiori, 80011 Acerra, Italy
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Mount Sinai School of Medicine, New York, NY 10029, USA
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Emergency Division, AORN Moscati, 83100 Avellino, Italy
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Department of Cardiology, University ‘L Vanvitelli’—Monaldi Hospital, 80131 Naples, Italy
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Division of Allergy and Clinical Immunology, Department of Medicine, University of Salerno, 84121 Salerno, Italy
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Rheumatology Unit, Department of Clinical Medicine and Surgery, University Federico II, 80131 Naples, Italy
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Cardiology, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, 35128 Padua, Italy
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Hematology and Clinical Immunology, Department of Medicine, University of Padova, 35128 Padova, Italy
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Cardiovascular Pathology, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, 35128 Padova, Italy
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Pediatric Cardiology, University ‘L Vanvitelli’—Monaldi Hospital, 8013 Naples, Italy
*
Author to whom correspondence should be addressed.
These authors contributed equally to this work.
Academic Editor: Matteo Vatta
Cardiogenetics 2022, 12(2), 133-141; https://doi.org/10.3390/cardiogenetics12020014
Received: 3 January 2022 / Revised: 10 March 2022 / Accepted: 22 March 2022 / Published: 28 March 2022
(This article belongs to the Special Issue Cardiogenetics: Feature Papers 2022)
Eosinophilic pancarditis (EP) is a rare, often unrecognized condition caused by endomyocardial infiltration of eosinophil granulocytes (referred as eosinophilic myocarditis, EM) associated with pericardial involvement. EM has a variable clinical presentation, ranging from asymptomatic cases to acute cardiogenic shock requiring mechanical circulatory support (MCS) or chronic restrictive cardiomyopathy at high risk of progression to dilated cardiomyopathy (DCM). EP is associated with high in-hospital mortality, particularly when associated to endomyocardial thrombosis, coronary arteries vasculitis or severe left ventricular systolic dysfunction. To date, there is a lack of consensus about the optimal diagnostic algorithm and clinical management of patients with biopsy-proven EP. The differential diagnosis includes hypersensitivity myocarditis, eosinophil granulomatosis with polyangiitis (EGPA), hypereosinophilic syndrome, parasitic infections, pregnancy-related hypereosinophilia, malignancies, drug overdose (particularly clozapine) and Omenn syndrome (OMIM 603554). To our knowledge, we report the first case of pancarditis associated to eosinophilic granulomatosis with polyangiitis (EGPA) with negative anti-neutrophil cytoplasmic antibodies (ANCA). Treatment with steroids and azathioprine was promptly started. Six months later, the patient developed a relapse: treatment with subcutaneous mepolizumab was added on the top of standard therapy, with prompt disease activity remission. This case highlights the role of a multimodality approach for the diagnosis of cardiac involvement associated to systemic immune disorders. View Full-Text
Keywords: eosinophilic myocarditis; eosinophilic granulomatosis with polyangiitis; pancarditis eosinophilic myocarditis; eosinophilic granulomatosis with polyangiitis; pancarditis
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MDPI and ACS Style

Lioncino, M.; Monda, E.; Dellegrottaglie, S.; Cirillo, A.; Caiazza, M.; Fusco, A.; Esposito, F.; Verrillo, F.; Ciccarelli, G.; Rubino, M.; Triggiani, M.; Scarpa, R.; Caforio, A.L.P.; Marcolongo, R.; Rizzo, S.; Basso, C.; Nigro, G.; Russo, M.G.; Golino, P.; Limongelli, G. Pancarditis as the Clinical Presentation of Eosinophilic Granulomatosis with Polyangiitis: A Multimodality Approach to Diagnosis. Cardiogenetics 2022, 12, 133-141. https://doi.org/10.3390/cardiogenetics12020014

AMA Style

Lioncino M, Monda E, Dellegrottaglie S, Cirillo A, Caiazza M, Fusco A, Esposito F, Verrillo F, Ciccarelli G, Rubino M, Triggiani M, Scarpa R, Caforio ALP, Marcolongo R, Rizzo S, Basso C, Nigro G, Russo MG, Golino P, Limongelli G. Pancarditis as the Clinical Presentation of Eosinophilic Granulomatosis with Polyangiitis: A Multimodality Approach to Diagnosis. Cardiogenetics. 2022; 12(2):133-141. https://doi.org/10.3390/cardiogenetics12020014

Chicago/Turabian Style

Lioncino, Michele, Emanuele Monda, Santo Dellegrottaglie, Annapaola Cirillo, Martina Caiazza, Adelaide Fusco, Francesca Esposito, Federica Verrillo, Giovanni Ciccarelli, Marta Rubino, Massimo Triggiani, Raffaele Scarpa, Alida L.P. Caforio, Renzo Marcolongo, Stefania Rizzo, Cristina Basso, Gerardo Nigro, Maria G. Russo, Paolo Golino, and Giuseppe Limongelli. 2022. "Pancarditis as the Clinical Presentation of Eosinophilic Granulomatosis with Polyangiitis: A Multimodality Approach to Diagnosis" Cardiogenetics 12, no. 2: 133-141. https://doi.org/10.3390/cardiogenetics12020014

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