Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis characterized by asthma, eosinophilia, and diffuse eosinophilic infiltration. Although cardiovascular involvement is common and a leading cause of EGPA-related mortality, severe pericarditis-led cardiac tamponade occurs rarely. We herein report a 72-year-old man with anti-proteinase 3 (anti-PR3) anti-neutrophil cytoplasmic antibody (ANCA)-positive EGPA diagnosed by the presence of cardiac tamponade, which responded quickly to pericardiocentesis and a single administration of prednisolone. This is the first case of anti-PR3 ANCA-positive EGPA with cardiac tamponade; the patient displayed clinical features of both ANCA-positive and ANCA-negative cases.
Keywords: Churg-Strauss syndrome; anti-proteinase 3 anti-neutrophil cytoplasmic antibodies; cardiac tamponade; eosinophilic granulomatosis with polyangiitis; pericarditis.