Granulomatosis con poliangeítis: actualización y conceptos claves

Simón Hoyos Patiño

Granulomatosis con poliangeítis: actualización y conceptos claves

Simón Hoyos Patiño

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Resumen

La granulomatosis con poliangeítis es una enfermedad multisistémica que hace parte de las vasculitis de pequeños vasos asociadas a ANCA (anticuerpos contra el citoplasma de los neutrófilos), junto con la granulomatosis eosinofílica con poliangeítis, la poliangeítis microscópica y la vasculitis asociada a ANCA limitada a un órgano. Esta patología se caracteriza por ser una vasculitis necrotizante, inflamatoria, con formación de granulomas. Afecta principalmente el tracto respiratorio y el sistema renal, con una mayor incidencia entre los 40-55 años. Tiene una mayor prevalencia en caucásicos y en el norte de Europa. Su etiología es desconocida, sin embargo en su aparición intervienen diversos aspectos ambientales, infecciosos, farmacológicos, inmunológicos y tóxicos, sumados a una predisposición genética. El objetivo del presente texto es hacer una revisión de los aspectos claves de la enfermedad con base a los nuevos avances en la literatura, incluyendo aspectos como la epidemiología, fisiopatología, manifestaciones clínicas, diagnóstico y tratamiento. Haciendo especial énfasis en la fisiopatología y en las manifestaciones clínicas, los cuales son fundamentales para el desarrollo de nuevas terapias para el manejo de la enfermedad, junto con un diagnóstico oportuno que permita limitar al máximo las complicaciones derivadas de esta.

Palabras clave

Granulomatosis con poliangeítis; vasculitis; ANCA; NETs.

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