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抗中性粒细胞胞浆抗体相关性血管炎早期和晚期肾脏结局的组织学及临床预测因素

Histological and clinical predictors of early and late renal outcome in ANCA-associated vasculitis.

作者信息

Neumann Irmgard, Kain Renate, Regele Heinz, Soleiman Afschin, Kandutsch Sandra, Meisl Franz Thomas

机构信息

Department of Nephrology, Wilhelminenspital, Vienna, Austria.

出版信息

Nephrol Dial Transplant. 2005 Jan;20(1):96-104. doi: 10.1093/ndt/gfh563. Epub 2004 Nov 16.

Abstract

BACKGROUND

Renal involvement remains a major determinant in antineutrophil cytoplasmic autoantibody-associated small vessel vasculitis (AASV). While some patients may develop persistent renal damage, others have a favourable outcome.

METHODS

To identify patients at risk for poor renal outcome, we evaluated 95 renal biopsies (67 initial biopsies and 28 repeat biopsies) of 67 patients with AASV for the presence and extent of active (AI) and chronic (CI) lesions, retrospectively. AI, CI, levels of proteinuria and dose of cyclophosphamide (CYC) were related to renal outcome.

RESULTS

Recovery of renal function in patients initially dialysis dependent was associated with a lower CI compared with patients who remained on dialysis (P<0.001), while AI did not differ significantly. In these patients, age <65 years revealed a positive predictive value of 85% for renal function recovery. Patients initially requiring dialysis exhibited a higher AI and CI compared with those who did not. Renal function in long-term follow-up correlated with CI and the amount of proteinuria. This relationship increased with time, exhibiting at 4 years a correlation coefficient of 0.607 for CI (P<0.01) and of 0.775 for proteinuria (P<0.001). Follow-up biopsies showed a more pronounced CI compared with initial biopsies (P<0.001). None of the investigated initial parameters was predictive for renal relapse. However, there was a relationship between dose and duration of CYC and time to relapse. Compared with the initial biopsy, repeat biopsies of eight patients with a creeping serum creatinine in clinical remission showed a decrease of AI (P<0.001) while CI increased rapidly. These patients also had less initial CYC (NS).

CONCLUSIONS

These data suggest that in AASV, evaluation of renal histopathology is helpful in predicting early and late renal outcome. Chronicity and proteinuria were the best determinants of poor renal prognosis. Activity may regress under therapy, while chronicity may progress despite treatment. The amount of CYC seems to influence the occurrence of early relapses and renal survival.

摘要

背景

肾脏受累仍是抗中性粒细胞胞浆抗体相关性小血管炎(AASV)的主要决定因素。虽然一些患者可能会出现持续性肾脏损害,但其他患者预后良好。

方法

为了识别肾脏预后不良的风险患者,我们回顾性评估了67例AASV患者的95份肾活检标本(67份初次活检标本和28份重复活检标本),以确定活动性(AI)和慢性(CI)病变的存在及程度。AI、CI、蛋白尿水平和环磷酰胺(CYC)剂量与肾脏预后相关。

结果

与仍需透析的患者相比,最初依赖透析的患者肾功能恢复与较低的CI相关(P<0.001),而AI无显著差异。在这些患者中,年龄<65岁对肾功能恢复的阳性预测值为85%。最初需要透析的患者与不需要透析的患者相比,AI和CI更高。长期随访中的肾功能与CI和蛋白尿量相关。这种关系随时间增加,在4年时CI的相关系数为0.607(P<0.01),蛋白尿的相关系数为0.775(P<0.001)。随访活检显示,与初次活检相比,CI更明显(P<0.001)。所研究的初始参数均不能预测肾脏复发。然而,CYC的剂量和持续时间与复发时间之间存在关系。与初次活检相比,临床缓解期血清肌酐缓慢升高的8例患者的重复活检显示AI降低(P<0.001),而CI迅速增加。这些患者最初使用的CYC也较少(无统计学意义)。

结论

这些数据表明,在AASV中,评估肾脏组织病理学有助于预测早期和晚期肾脏预后。慢性病变和蛋白尿是肾脏预后不良的最佳决定因素。活动性病变在治疗下可能消退,而慢性病变可能尽管接受治疗仍会进展。CYC的用量似乎会影响早期复发的发生和肾脏存活情况。

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