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ANCA-associated vasculitis: diagnostic and therapeutic strategy Ozaki SAllergol Int 2007[Jun]; 56 (2): 87-96Among small-vessel vasculitides, microscopic polyangiitis (MPA), Wegener's granulomatosis (WG), and allergic granulomatous angiitis (AGA) are known collectively as ANCA-associated vasculitis (AAV) because of the involvement of anti-neutrophil cytoplasmic antibodies (ANCA) as the common pathogenesis. Major target antigens of ANCA associated with vasculitis are myeloperoxidase (MPO) and proteinase 3 (PR3). MPO-ANCA is related to MPA and AGA, and PR3-ANCA is the marker antibody in WG. MPO-ANCA-associated vasculitis is more frequent in Japan, whereas PR3-ANCA-associated vasculitis is more common in Europe and USA. ANCA appears to induce vasculitis by directly activating neutrophils. Therefore, no immunoglobulins or complement components are detected in the vasculitis lesions; hence, AAV is called pauci-immune vasculitis (pauci = few/little). Untreated patients with severe AAV with multi-organ involvement have a poor prognosis, which is improved by combination therapy with cyclophosphamide and high-dose corticosteroid. Randomized controlled trials (RCT) regarding induction and maintenance of remission of AAV indicated that the rate of remission induction by the standard regimen is approximately 90% in 6 months, that maintenance of remission can be achieved with oral azathioprine as well as cyclophosphamide, and that methotrexate can be used only for non-renal mild AAV. As these data were obtained mostly in patients positive for PR3-ANCA, caution must be taken in applying these findings to Japanese patients, most of whom are positive for MPO-ANCA. A prospective study is now underway to clarify the effectiveness of the standard regimen in Japanese patients with MPO-ANCA-associated vasculitis. This article describes the diagnostic criteria and the recent evidence-based therapeutic strategy of AAV.|Adrenal Cortex Hormones/therapeutic use[MESH]|Antibodies, Antineutrophil Cytoplasmic/*blood[MESH]|Azathioprine/therapeutic use[MESH]|Biological Products/therapeutic use[MESH]|Churg-Strauss Syndrome/diagnosis/enzymology/immunology/*therapy[MESH]|Cyclophosphamide/therapeutic use[MESH]|Drug Therapy, Combination[MESH]|Granulomatosis with Polyangiitis/diagnosis/enzymology/immunology/*therapy[MESH]|Humans[MESH]|Immunoglobulins, Intravenous/therapeutic use[MESH]|Immunologic Factors/*therapeutic use[MESH]|Immunosuppressive Agents/therapeutic use[MESH]|Methotrexate/therapeutic use[MESH]|Myeloblastin/immunology[MESH]|Peroxidase/immunology[MESH]|Remission Induction[MESH]|Severity of Illness Index[MESH]|Treatment Outcome[MESH]|Vasculitis/diagnosis/enzymology/immunology/*therapy[MESH] |
