A life-threatening presentation of eosinophilic granulomatosis with polyangiitis

• Published in: Journal of cardiovascular medicine (Hagerstown, Md.) Vol. 17 Suppl 2; p. e109
• Main Authors: Ammirati, Enrico, Cipriani, Manlio, Musca, Francesco, Bonacina, Edgardo, Pedrotti, Patrizia, Roghi, Alberto, Astaneh, Arash, Schroeder, Jan W, Nonini, Sandra, Russo, Claudio F, Oliva, Fabrizio, Frigerio, Maria
• Format: Journal Article
• Language: English
• Published: United States 01.12.2016
• Subjects: Administration, Intravenous; Adult; Biopsy; Churg-Strauss Syndrome - complications; Churg-Strauss Syndrome - diagnosis; Churg-Strauss Syndrome - therapy; Extracorporeal Membrane Oxygenation; Granulomatosis with Polyangiitis - complications; Granulomatosis with Polyangiitis - diagnosis; Granulomatosis with Polyangiitis - therapy; Humans; Immunosuppressive Agents - administration & dosage; Magnetic Resonance Imaging; Male; Myocarditis - diagnosis; Myocarditis - etiology; Myocarditis - therapy; Myocardium - pathology; Necrosis; Shock, Cardiogenic - diagnosis; Shock, Cardiogenic - etiology; Shock, Cardiogenic - therapy; Steroids - administration & dosage; Time Factors; Treatment Outcome
• ISSN: 1558-2035
• DOI: 10.2459/JCM.0000000000000330

: Necrotizing eosinophilic myocarditis (NEM) is a life-threatening condition that needs rapid diagnosis by endomyocardial biopsy and hemodynamic support usually by mechanical circulatory systems. We present the case of a 25-year-old Caucasian man who developed a refractory cardiogenic shock due to a NEM that was supported with a peripheral veno-arterial extracorporeal membrane oxygenation associated with intravenous steroids and recovered after 2 weeks. Further instrumental investigations lead to the final diagnosis of NEM as first presentation of eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome), remarking the importance of identifying the systemic disorder that usually triggers the eosinophilic damage of the myocardium.