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Antes Revista del Instituto Nacional de Enfermedades Respiratorias

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2017, Número 1

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Neumol Cir Torax 2017; 76 (1)


Granulomatosis eosinofílica con poliangitis: reporte de un caso y revisión de la literatura

Rodríguez-Armendáriz R, Hernández-Saldaña R, Hinojos-Gallardo LC, Ramos-Martínez E, Soto-Ramos M

Idioma: Español
Referencias bibliográficas: 49
Paginas: 36-43
Archivo PDF: 409.54 Kb.


RESUMEN

La granulomatosis eosinofílica con poliangitis (GEPA), anteriormente denominado síndrome de Churg-Strauss (SCS), es un trastorno poco frecuente, de etiología desconocida, caracterizada por una vasculitis necrosante que afecta a vasos de pequeño a mediano calibre. Los pacientes presentan como rasgo característico antecedentes de asma, rinitis alérgica y eosinofilia en sangre periférica. La GEPA, antes denominado SCS es poco común en la infancia. Aunque la GEPA es una vasculitis asociada a la presencia de anticuerpos anticitoplasma de neutrófilo (ANCA), en niños sólo se encuentran en menos del 40% de los casos. La detección temprana de la enfermedad es importante ya que un retraso en el diagnóstico puede llevar a la afectación orgánica de grado variable con resultados fatales. La GEPA de inicio en la infancia representa menos del 2% de los casos de vasculitis en edad pediátrica, y sólo 50 casos de GEPA en menores de 18 años se habían publicado hasta el año 2013. Presentamos el caso de un niño de 13 años de edad con asma de difícil control y deterioro de su estado general, que finalmente fue diagnosticado con GEPA-ANCA negativo.


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