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首页> 外文期刊>Current opinion in hematology >Pathophysiological importance of antineutrophi! antibodies in vasculitis
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Pathophysiological importance of antineutrophi! antibodies in vasculitis

机译：抗中性粒细胞的病理生理重要性！血管炎中的抗体

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Purpose of review We endeavour to provide a brief overview of the recent advances in understanding of how antineutrophil cytoplasmic antibodies (ANCAs) contribute to the pathophysiology of vasculitis. Recent findings Substantial progress has been made in our understanding of the immunopathogenesis of ANCA-associated vasculitides. Compelling evidence from in-vitro studies and experimental models in conjunction with clinical trials has confirmed that ANCAs directly contribute to the evolution and progression of the disease process. A new ANCA, directed against human lysosome membrane protein-2 (LAMP-2), has recently been described as a sensitive and specific marker for renal vasculitis and we discuss its potential impact for diagnosis and therapy. Furthermore, high-throughput approaches are starting to identify genetic patterns that may identify patients likely to respond to specific therapy or having a high probability of relapse. Summary It has become increasingly clear over the last two decades that ANCA IgG is pathogenic in vasculitis. Novel therapies aimed at selected cell populations or blocking specific pathogenic pathways offer hope for more selectively treating this heterogeneous group of patients, while avoiding nonspecific immunosuppression and its adverse effects.
机译：综述的目的我们努力简要概述抗中性粒细胞胞浆抗体（ANCA）如何促进血管炎的病理生理学的最新进展。最近的发现在我们对ANCA相关血管炎的免疫发病机理的理解上取得了实质性进展。来自体外研究和实验模型以及临床试验的令人信服的证据已经证实，ANCA直接促进了疾病过程的发展。最近，针对人溶酶体膜蛋白2（LAMP-2）的新型ANCA被描述为肾血管炎的敏感和特异性标志物，我们讨论了其对诊断和治疗的潜在影响。此外，高通量方法已开始鉴定可识别可能对特定疗法有反应或复发可能性高的患者的遗传模式。总结在过去的二十年中，ANCA IgG在血管炎中具有致病性，这一点变得越来越清楚。针对选定的细胞群或阻断特定病原体途径的新疗法为更选择性地治疗这一异类患者提供了希望，同时避免了非特异性免疫抑制及其不利影响。

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