Published online Sep 26, 2020. doi: 10.4330/wjc.v12.i9.460
Peer-review started: July 2, 2020
First decision: June 20, 2020
Revised: July 2, 2020
Accepted: September 1, 2020
Article in press: September 1, 2020
Published online: September 26, 2020
Eosinophilic granulomatosis polyangiitis (EGPA) is a small vessel necrotizing vasculitis that commonly presents as peripheral eosinophilia and asthma; however, it can rarely manifest with cardiac involvement such as pericarditis and cardiac tamponade. Isolated pericardial tamponade presenting as the initial symptom of EGPA is exceedingly rare. Early diagnosis and appropriate treatment are crucial to prevent life-threatening outcomes.
52-year-old woman with no past medical history presented with progressive dyspnea and dry cough. On physical exam she had a pericardial friction rub and bilateral rales. Vital signs were notable for tachycardia at 119 beats per minute and hypoxia with 89% oxygen saturation. On laboratory exam, she had 45% peripheral eosinophilia, troponin elevation of 1.1 ng/mL and N-terminal prohormone of brain natriuretic peptide of 2101 pg/mL. TTE confirmed a large pericardial effusion and tamponade physiology. She underwent urgent pericardial window procedure. Pericardial and lung biopsy demonstrated eosinophilic infiltration. Based on the American College of Radiology guidelines, the patient was diagnosed with EGPA which manifested in its rare form of cardiac tamponade. She was treated with steroid taper and mepolizumab.
This case highlights that when isolated pericardial involvement occurs in EGPA, diagnosis is recognized by performing pericardial biopsy demonstrating histopathologic evidence of eosinophilic infiltration.
Core Tip: (1) To be able to investigate the etiology of pericardial effusion and cardiac tamponade with eosinophilia which is rarely caused by eosinophilic granulomatosis polyangiitis (EGPA); (2) To be mindful that anti-neutrophil cytoplasmic antibody is negative in EGPA with cardiac involvement rather than pulmonary or renal involvement; (3) To be aware that when isolated pericardial involvement leading to cardiac tamponade occurs, diagnosis is recognized by performing pericardial biopsy demonstrating histopathologic evidence of eosinophilic infiltration; (4) To consider early diagnosis of EGPA with cardiac involvement is crucial because it carries a major burden of morbidity and mortality; (5) To initiate early treatment with corticosteroids when an isolated pericardial involvement is present whereas immunosuppressants are utilized with multiorgan involvement; and (6) To conduct close surveillance in the outpatient setting to monitor the response to treatment and maintenance medications such as steroids and monoclonal antibodies.