Resumen
Introducción
Las vasculitis necrotizantes son enfermedades inflamatorias sistémicas de etiología desconocida, potencialmente graves, que cursan con recaídas frecuentes y que afectan principalmente a vasos de mediano y pequeño calibre. Sin tratamiento se asocian con una elevada morbimortalidad.
TratamientoEl tratamiento se basa en la administración de glucocorticoides, habitualmente en combinación con un inmunosupresor, habiéndose establecido distintas pautas terapéuticas en función de su gravedad y extensión en el momento del diagnóstico. La terapia biológica, especialmente con rituximab, es una alternativa terapéutica eficaz.
Evolución y pronósticoExisten distintas escalas que permiten estimar la actividad, el pronóstico y el daño residual de dichas vasculitis. La supervivencia de los pacientes afectos de vasculitis necrotizantes ha mejorado gracias a un diagnóstico más precoz y a la estratificación del tratamiento.
Palabras clave
Panarteritis nodosa
Poliangitis microscópica
Poliangitis microscópica con granulomatosis
Granulomatosis eosinofílica con poliangitis
Kawasaki
Abstract
Introduction
Necrotising vasculitis are systemic inflammatory diseases of unknown aetiology, potentially severe, that develop with frequent relapses and affect small and medium sized vessels. If untreated they are associated to a high morbidity and mortality.
TreatmentTreatment is based in administering glucocorticoids, mostly in combination with an immunosuppression agent, establishing different therapeutic approaches depending on severity and extension at the time of diagnosis. Biological therapy, specially with rituximab, is an efficient alternative.
Evolution and prognosisThere are different scales that allow to assess activity, prognosis and residual damage of these vasculitis. Survival of patients with necrotising vasculitis has improved thanks to an earlier diagnosis and the treatment stratification.
Keywords
Panarteritis nodosa
Microscopic polyangiitis
Microscopic polyangiitis with granulomatosis
Eosinophilic Granulomatosis with Polyangiitis
Kawasaki
Identifíquese
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