Unexpected death due to eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
DOI:
https://doi.org/10.23938/ASSN.0395Keywords:
Churg-Strauss syndrome. Eosinophilic granulomatosis with polyangiitis. Hipereosinophilia. Vasculitis.Abstract
Eosinophilic granulomatosis with polyangiitis (EGPA), or Churg-Strauss syndrome, is a rare disease characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring among patients with asthma and tissue eosinophilia. Clinically, it presents in various ways and progresses in three phases: prodromic (asthma and rhino-sinusitis), eosinophilic (peripheral eosinophilia and organ involvement), and vasculitic (clinical manifestations due to small vessel vasculitis). The differential diagnosis of EGPA principally includes eosinophilic and vasculitic disorders, early treatment is needed to avoid a fatal outcome.
We present the case report of a 38-year-old male with a history of asthma. After a month-and-a-half of progressive weakness, no fever, diarrhea, vomiting and abdominal pain associated with weight loss, he was diagnosed of intestinal parasitosis. He later died of a massive myocardial necrosis due to EGPA with multiple organs affected.
Downloads
References
GRECO A, RIZZO MI, DE VIRGILIO A, GALLO A, FUSCONI M, RUOPPOLO G et al. Churg-Strauss syndrome. Autoimmun Rev 2015; 14: 341-348. https://doi.org/10.1016/j.autrev.2014.12.004
HEMMETT J, QIRJAZI E, WEIR MA, MOUSSA M, LANDRY YD, GUNARATNAM L. Cardiac, renal, and central nervous system dysfunction with eosinophilia: Eosinophilic granulomatosis with polyangiitis. Lancet 2015; 385: 480. https://doi.org/10.1016/S0140-6736(14)62352-7
PACHECO CUADROS R, SENDINO REVUELTA A, MORALES BASTOS C, GÓMEZ CEREZO J, VÁZQUEZ RODRÍGUEZ JJ, BARBADO HERNÁNDEZ FJ. Vasculitis necrosante sistémica en la autopsia. Rev Clin Esp 2001; 201; 378-384.
MASI AT, HUNDER GG, LIE JT, MICHEL BA, BLOCH DA, AREND WP et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990; 33: 1094-1100. https://doi.org/10.1002/art.1780330806
VAL-BERNAL JF, MAYORGA M, GARCÍA-ALBERDI E, POZUETA JA. Churg-Straus syndrome and sudden cardiac death. Cardiovasc Path 2003; 12: 93-97. https://doi.org/10.1016/S1054-8807(02)00157-6
GUILLEVIN L, COHEN P, GAYRAND M, LHOTE F, JARROUSSE B, CASASSUS P. Churg-Strauss syndrome. Medicine 1999; 78: 28-37.
DOMÍNGUEZ F, KÜHL U, PIESKE B, GARCIA-PAVIA P, TSCHÖPE C. Actualización sobre miocarditis y miocardiopatía inflamatoria: el resurgir de la biopsia endomiocárdica. Rev Esp Cardiol 2016; 69: 178-187. https://doi.org/10.1016/j.recesp.2015.10.018
GROH M, PAGNOUX C, BALDINI C, BEL E, BOTTERO P, COTTIN V et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Intern Med 2015; 26: 545-553. https://doi.org/10.1016/j.ejim.2015.04.022
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2019 Anales del Sistema Sanitario de Navarra
This work is licensed under a Creative Commons Attribution-ShareAlike 4.0 International License.
La revista Anales del Sistema Sanitario de Navarra es publicada por el Departamento de Salud del Gobierno de Navarra (España), quien conserva los derechos patrimoniales (copyright ) sobre el artículo publicado y favorece y permite la difusión del mismo bajo licencia Creative Commons Reconocimiento-CompartirIgual 4.0 Internacional (CC BY-SA 4.0). Esta licencia permite copiar, usar, difundir, transmitir y exponer públicamente el artículo, siempre que siempre que se cite la autoría y la publicación inicial en Anales del Sistema Sanitario de Navarra, y se distinga la existencia de esta licencia de uso.