AAzad
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Antineutrophil cytoplasmic antibodies (ANCA) are autoantibodies observed in patient serum in a wide range of vasculitic diseases including Wegener's granulomatosis, Churg-Strauss syndrome, idiopathic crescentic glomerulonephritis, and microscopic polyangiitis where a renal-pulmonary syndrome including pauci-immune necrotizing crescentic glomerulonephritis (NCGN) and pulmonary capillaritis can be observed .
Proteinase 3 and myeloperoxidase (MPO), both myeloid lysosomal enzymes, have been identified as the primary target antigens for ANCA in those disorders . It has been well established that detection of ANCA is useful in the diagnosis and monitoring of systemic vasculitic disorders . However, the pathophysiological role of ANCA in their associated diseases has not been fully elucidated. In vitro experiments have shown that ANCA can activate neutrophils leading to oxygen radical production and degranulation of proteolytic lysosomal enzymes . In addition, ANCA promote the adhesion of neutrophils to monolayers of endothelial cells and may cause vascular injury by activating primed neutrophils to induce endothelial cell lysis . Although in vitro observations suggest a pathogenic role for ANCA, their in vivo relevance is not clear. Studying the pathophysiological role of ANCA in vivo reported on an animal model for anti-MPO-associated pauci-immune NCGN. pauci-immune NCGN is induced in MPO-immunized rats by renal perfusion of MPO, proteolytic enzymes, and H2O2 .
In most cases of ANCA-associated vasculitides, disease manifestations are not confined to the kidneys . Especially pulmonary involvement is frequently reported . In ANCA-associated vasculitis a similar pathogenesis may underlie the disease manifestations in both lungs and kidneys.
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AAzad
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| anjushree
Forum Guru
Topics: 64
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CMDT----MCC of renal pul syndrome is microscopic polyangiitis
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| prathapdoctor
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is it, thank you for putting forward a very good point
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