中文摘要：

目的：观察双重血浆置换（doublefiltrationplasmapheresis，DFPP）治疗抗中性粒细胞胞质抗体（ANCA）相关血管炎（ANCAassociatedvasculitis，AAV）的初步临床疗效。方法：12例活动期AAV患者（男7例，女5例，年龄43～72岁），BVAS评分14．7±3．2分，其中10例p／MPO—ANCA阳性，2例c／PR3-ANCA阳性，ANCA水平221．3—341．4RU／ml（平均274．9±33．9RU／m1）。平均血清肌酐（SCr）549．9±197．1μmol／L，临床表现均符合急进性肾炎综合征（RPGN），均需要肾脏替代治疗。肾活检病理类型分为新月体型（细胞性新月体≥50％，5例），混合型（细胞性新月体〈50％，肾小球硬化〈50％，3例），局灶型（I〉50％肾小球正常，1例）和硬化型（≥50％肾小球硬化，3例）。DFPP采用血浆分离器EC50W作一级滤器，血浆成分分离器EC20W作二级滤器，每次处理血浆量为2倍血浆容量。均同时给予甲泼尼松静脉治疗（0．5g／d，3—6d），后续采用激素联合吗替麦考酚酯或间断静脉环磷酰胺冲击治疗。观察DFPP治疗前后血清ANCA水平变化和对肾功能的影响。结果：DFPP治疗3～5次，治疗结束时ANCA水平显著下降[（274．9土33．9）RU／ml vs（111．8±55．1）RU／ml，P〈0．01]，平均下降（60．0±18．1）％。10例（83．3％）摆脱透析，1例（新月体型）仅随访1月，尚未摆脱透析，1例（硬化型）肾功能无改善，转为维持性肾脏替代治疗。摆脱透析的10例患者平均随访6．2±4．8月（1～15月），1例（局灶型）肾功能恢复正常，8例（新月体型和混合型各3例，硬化型2例）SCr稳定，1例（新月体型）1月后SCr再次升高行肾脏替代治疗。DFPP治疗过程中无明显不良反应。结论：DFPP能有效清除AAV患者血清ANCA，改善。肾功能，但其疗效还需大样本临床对照试验研究的证实。

英文摘要：

Objective： To investigate the clinical efficacy of double filtration plasmapheresis （DFPP） in antineutrophil cytoplasmic autoantibody （ANCA） associated vasculitis （AAV） with severe renal involvement. Methodology： Twelve patients （female 5, male 7, age 43 - 72 years ） with new onset and active AAV were included this study. BVAS scores were （14. 7 ±3.2）. 10 patients had serum positive P/MPO-ANCA,the others had serum positive C/ PR3-ANCA, and the average levels of ANCA were （275 ± 33.9 ） RU/ml. On admission, they had a mean SCr of （ 549 ± 197 ） μmol/L and all of them needed renal replacement therapy. Every case had renal biopsy, and histological patterns were classified into focal （ ≥ 50% normal glomeruli, n = 1 ）, crescentic （ ≥50% glomeruli with cellular crescents, n = 5 ）, mixed （ 〈 50% crescentic and 〈 50% globally sclerotic glomeruli, n = 3 ） and sclerotic （≥50% globally sclerotic glomeruli, n = 3 ）. All of them were received DFPP treatment as well as methylprednisolone pulse therapy followed by oral prednisone and mycophenloate mofetil （MMF） or intravenous cyclophosphamide pulse therapy. Double volumes of plasma were processed during each DFPP session every other day. ECSOW and EC20W （ Asahi Kasei Corporation, Japan） were used as first filter for plasma separation and second filter for plasma fractionation, respectively. The changes of ANCA and renal function were investigated. Results： All patients were treated with DFPP for 3 - 5 times. At the end of DFPP, the serum level of MPO- ANCA from [ （275 ± 33.9） to （ 1 112± 55. 1 ） RU/ml, P 〈 0. 01 ] was lower significantly, with a decrement of （ 60. 0 ± 18.1 ）%. Ten patients （83.3%） were free from dialysis, while one patient （the pathologic classification was crescentic） was followed only one month have not been free from dialysis, the other one without improvement of renal function received maintenance hemodialysis. Ten patients who free from dialysis after DFPP were f