Abstract
ANCA (anti-neutrophil cytoplasmic antibodies) associated vasculitis or AAV are a group of diseases with predominant inflammation of small vessels and the presence of detectable ANCA in serum. Due to these common features, it is considered that AAV share pathogenic mechanisms. Consequently, a similar therapeutic approach has been developed. A new nomenclature has been recently proposed, with AAV including "granulomatosis with polyangiitis" (GPA, formerly Wegener's granulomatosis), "microscopic polyangiitis" (MPA), "eosinophilic granulomatosis with polyangiitis" (EGPA, formerly Churg-Strauss disease) and renal limited vasculitis. Research on AAV has shown significant advances in the last two decades, including advances in the knowledge of pathogenic mechanisms such as the pro-inflammatory role of ANCA, AAV murine models and genetic links. Evidence on previous immunosuppressive therapies has improved significantly and new, promissory drugs have been introduced, Rituximab being the most important. We review the advances of pathogenic mechanisms and treatment for these diseases.
Highlights
anti-citoplasma de neutrófilos (ANCA) associated vasculitis or AAV are a group of diseases with predominant inflammation of small vessels and the presence of detectable ANCA in serum
Múltiple evidencia apoya el papel de los ANCA en la patogenia de las vasculitis asociadas a ANCA (VAA), destacando el desarrollo de modelos murinos de la enfermedad basados en los antígenos más relevantes, PR3 y MPO
Cartin-Ceba R, Golbin J, Keogh K, Peikert T, Fervenza F, Ytterberg S, et al Rituximab for Remision Induction and Maintenance in anti-citoplasma de neutrófilos (ANCA)-Associated Vasculitis: A Single-Center Ten Year Experience in 108 patients Arthritis & Rheumatism 2010; 62 (Supplement 10): S 283
Summary
Vasculitis asociadas a anticuerpos anti-citoplasma de neutrófilos: avances en patogenia y tratamiento. ANCA (anti-neutrophil cytoplasmic antibodies) associated vasculitis or AAV are a group of diseases with predominant inflammation of small vessels and the presence of detectable ANCA in serum. Due to these common features, it is considered that AAV share pathogenic mechanisms. Fuente de apoyo financiero: Concurso INICIO No 33/2010 VRAID, Pontificia Universidad Católica de Chile. La frecuente presencia de los anticuerpos anti-citoplasma de neutrófilos (ANCA) y el compromiso inflamatorio de la pared de vasos pequeños en múltiples órganos son las características centrales del grupo de vasculitis asociadas a ANCA (VAA). Acrónimo Inducción CYCLOPS NORAM RAVE RITUXVAS MEPEX Mantención CYCAZAREM IMPROVE MAINRITSAN
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