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Fulminant Eosinophilic Myocarditis: a rare initial presentation of Churg-Strauss Syndrome
Session:
Sessão de Casos Clínicos - II
Speaker:
Pedro M. Lopes
Congress:
CPC 2019
Topic:
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Session Type:
Sessão de Casos Clínicos
FP Number:
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Authors:
Pedro M. Lopes; Bruno Rocha; Gonçalo Lopes Da Cunha; Sara Ranchordás; Catarina Albuquerque; António Miguel Ferreira; Jorge Santos Ferreira; Carlos Aguiar; Marisa Trabulo; Sância Ramos; José Pedro Neves; Miguel Mendes
Abstract
<p><strong>Case Presentation: </strong></p> <p>A 22-year-old male presented to the emergency room with nausea, vomiting, anorexia and lumbar pain for 5 days. He also had chest pain prior to admission. His past history was notable for asthma and allergic rhinitis. Physical examination was unremarkable, other than a 126bpm regular tachycardia. Laboratory workup showed leukocytosis (24.0x10<sup>9</sup>/L), marked eosinophilia (11.7x10<sup>9</sup>/L, 48%) and elevated C-reactive protein (19.9mg/dL). Hs-Cardiac troponin T and NT-proBNP were increased, 2500ng/L and 18795pg/mL, respectively. There was inferolateral ST-segment depression on ECG and transthoracic echocardiogram revealed severe left ventricular (LV) systolic dysfunction due to global hypokinesis, a restrictive filling pattern and pericardial thickening. Chest CT revealed bilateral ground-glass lung infiltrates and paranasal CT scan showed cranial sinus opacifications.</p> <p> </p> <p>He rapidly progressed to cardiogenic shock in the following hours and was urgently transferred to a Cardiac Intensive Care Unit. Invasive ventilation, empiric antibiotics and IV inotropes were started. However, peripheral veno-arterial extracorporeal membrane oxygenation (ECMO) was required for hemodynamic support just 24h after admission. An endomyocardial biopsy was performed, revealing a necrotizing eosinophilic vasculitis and thrombosis. On further investigation, cardiotropic viruses, HIV, <em>Aspergillus</em>, <em>Toxoplasma</em> and parasites were negative, as were ANCA antibodies.</p> <p> </p> <p>Eosinophilic granulomatosis with polyangiitis (EGPA, or Churg-Strauss Syndrome) was considered as the most likely diagnosis, as four out of six criteria were present: (1) asthma; (2) eosinophilia; (3) extravascular eosinophils; and (4) paranasal sinusitis. Thus, IV corticosteroids were started, with significant improvement. ECMO was discontinued after 6 days, while being weaned off of both ventilator and inotropes. Cardiac MRI showed widespread subendocardial late gadolinium enhancement. Coronary arteries were unremarkable on CT-Angiography. Despite optimal medical treatment, LV ejection fraction plateaued at 34% and a subcutaneous ICD was implanted. After 45 days, the patient was doing well and was discharged home on oral glucocorticoids and twice monthly IV cyclophosphamide pulse treatment.</p> <p> </p> <p><strong>Conclusion</strong>: EGPA is a systemic necrotizing vasculitis often associated with respiratory findings and eosinophilia. We here report a rare case of rapidly progressing vasculitis presenting with fulminant myocardial involvement.</p>
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