Schmitt W H, Gross W L
Poliklinik für Rheumatologie, Medizinische Universität Lübeck und Rheumaklinik Bad Bramstedt, Germany.
Kidney Int Suppl. 1998 Feb;64:S39-44.
The clinical severity of ANCA-associated vasculitides (AAV) ranges widely from relatively benign courses to rapidly progressive, acutely life-threatening disease. The aggressiveness in the individual patient depends on the site and size of vessels involved and on the severity of the associated inflammatory/immunological features. The scope of life-threatening lesions reaches from the well-known pulmonary renal syndrome to cardiac, gastrointestinal and central nervous system manifestations. Treatment must be instituted as soon as possible and consists of daily cyclophosphamide and high-dose glucocorticosteroids. Plasmapheresis, intravenous immunoglobulin, and monoclonal antibodies may be of benefit in some cases.
抗中性粒细胞胞浆抗体相关血管炎（AAV）的临床严重程度差异很大，从相对良性的病程到快速进展、急性危及生命的疾病。个体患者的病情严重程度取决于受累血管的部位和大小以及相关炎症/免疫特征的严重程度。危及生命的病变范围从众所周知的肺肾综合征到心脏、胃肠道和中枢神经系统表现。必须尽快开始治疗，治疗方案包括每日使用环磷酰胺和大剂量糖皮质激素。在某些情况下，血浆置换、静脉注射免疫球蛋白和单克隆抗体可能有益。
