2021, Number 5
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Med Int Mex 2021; 37 (5)
Everything you should know about ANCAassociated small vessel vasculitis
Mercado U, Arzola E, Sepúlveda R
Language: Spanish
References: 25
Page: 827-833
PDF size: 207.63 Kb.
ABSTRACT
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides include granulomatosis
with polyangiitis (Wegener), microscopic polyangiitis, eosinophilic granulomatosis
with polyangiitis (Churg-Strauss), necrotizing and crescentic glomerulonephritis
that lacked well defined immunoglobulin deposit and drug-induced vasculitides. The
five phenotypes are characterized by small vessel vasculitis, absence or paucity of
vessel wall localization of immunoglobulin and complement, with or without granulomatous
inflammation, most often in the lung, and the presence of circulating ANCA
with specificity toward proteinase-3 or myeloperoxidase. The mechanism by which
ANCAs cause vasculitis is unknown. Experimental and clinical evidence suggest that
both PR-3 and MPO proteases are pathogenic. Traditionally, Fauci regimen of GPA
consists of daily oral cyclophosphamide and glucorticoids. Other therapies include
rituximab, plasmapheresis and mycophenolate.
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