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Anti-proteinase 3-positive Eosinophilic Granulomatosis with Polyangiitis Revealed by Cardiac Tamponade

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 occu...

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Detalles Bibliográficos
Autores principales: Yamamoto, Yuji, Otani, Yasushi, Okabe, Fukuko, Yoneda, Midori, Morimura, Osamu, Abe, Kinya
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6859378/
https://www.ncbi.nlm.nih.gov/pubmed/31243203
http://dx.doi.org/10.2169/internalmedicine.2937-19
Descripción
Sumario: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.