Abstract：
ANCA-associated Vasculitides (AAV) are characterized by small vessel necrotizing inflammation and can present with multisystem organ involvement, including organ/life threatening manifestations of rapidly progressive glomerulonephritis and diffuse alveolar haemorrhage, where immediate and aggressive intervention is needed to prevent further organ damage. Although, the rationale of plasma exchange (PLEX) in AAV is strong, through removing the pathogenic ANCAs; target either myeloperoxidase (MPO) or proteinase 3 (PR3), and other inflammatory molecules, especially in the initiation when the immunosuppressive treatment is no sufficient to prevent the organ damage, overall impact on patient outcomes is not well-established, while the risk of infections seems to be higher in the PLEX-treated patients. A comprehensive overview of the challenges and uncertainties surrounding the use of PLEX in the management of AAV will be reviewed, providing the current practice recommendations guiding treatment decisions.
摘要：
ANCA相关血管炎（AAV）的特点是小血管坏死性炎症，可表现为多系统器官受累，包括快速进展性肾小球肾炎和弥漫性肺泡出血的器官/危及生命的表现，需要立即积极干预以防止进一步的器官损伤。虽然,在AAV中血浆置换（PLEX）的基本原理是强大的，通过去除致病性ANCA；靶向髓过氧化物酶（MPO）或蛋白酶3（PR3），和其他炎症分子，特别是在开始免疫抑制治疗不足以防止器官损伤时，对患者预后的总体影响尚未确定，而PLEX治疗患者的感染风险似乎更高。将全面概述在AAV管理中使用PLEX的挑战和不确定性，提供当前的实践建议，指导治疗决策。
