An atypical presentation of cardiac tamponade and periorbital swelling in a patient with eosinophilic granulomatosis with polyangiitis: a case report

• Main Authors: Alexandra C. Keefe, Joseph C. Hymas, Lyska L. Emerson, John J. Ryan
• Format: Article
• Language: English
• Published: BMC 2017-09-01
• Series: Journal of Medical Case Reports
• Subjects: Churg-Strauss syndrome; Cardiac tamponade; Vasculitis; Periorbital swelling; Effusion
• Online Access: http://link.springer.com/article/10.1186/s13256-017-1434-9

Abstract Background Eosinophilic granulomatosis with polyangiitis is a rare, necrotizing systemic vasculitis associated with asthma and hypereosinophilia. Its cause and pathophysiology are still being elucidated. Case presentation We report a case of eosinophilic granulomatosis with polyangiitis in a 50-year-old Caucasian woman who presented with chest pain, dyspnea at rest, fever, and periorbital swelling. She was found to have significant hypereosinophilia and cardiac tamponade physiology. A biopsy confirmed extensive infiltration of both lungs and pericardium by eosinophils. She did not have any anti-neutrophil cytoplasmic antibodies. Conclusions Eosinophilic granulomatosis with polyangiitis diagnosis does not require the presence of anti-neutrophil cytoplasmic antibodies. Anti-neutrophil cytoplasmic antibody-positive and anti-neutrophil cytoplasmic antibody-negative eosinophilic granulomatosis with polyangiitis may present with different clinical phenotypes, perhaps suggesting two distinct disease etiologies and distinct pathophysiology.