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[抗中性粒细胞胞浆抗体相关血管炎中的快速进展性肾小球肾炎:病程、治疗疗效及预后]

[Rapidly progressive glomerulonephritis in ANCA-associated vasculitis: a course, treatment efficacy, prognosis].

作者信息

Tomilina N A, Biriukova L S, Egorova E T, Sukhanov A V, Stoliarevich E S, Kupavtseva O A, Fedorova N D, Frolov A V, Trushkin R N, Kurenkova L G

出版信息

Ter Arkh. 2008;80(6):15-24.

Abstract

AIM

To study efficacy of ANCA-RPGN treatment with corticosteroids and cyclophosphamide or mycophenolic acid drugs.

MATERIAL AND METHODS

We treated 28 patients (17 males and 11 females aged 19-71 years) with morphologically verified ANCA-associated crescentic RPGN (crescentic median 79 (63:88)%. The patients received corticosteroids and cytostatics.

RESULTS

The response to the treatment was registered in 22 (78%) patients in 8-16 weeks: a complete remission was achieved in 8 patients, a partial one--in 14 patients. In partial remission renal functions recovered incompletely (median Pcr 200 (180;255) mcmol/l) in persistence of moderate proteinuria (median 0.7 (0.6;1.3)g/day) and absence of microhematuria. Probability of the treatment success depended on severity of glomerulosclerosis and weakly depended on activity of extracapillary reaction. Severe renal failure was not an absolute predictor of treatment failure.

CONCLUSION

In the absence of advanced nephrosclerosis early treatment with corticosteroid in combination with cytostatics can produce a positive effect in 70-80% patients with ANCA associated RPGN.

摘要

目的

研究使用皮质类固醇和环磷酰胺或霉酚酸类药物治疗抗中性粒细胞胞浆抗体相关性急进性肾小球肾炎(ANCA-RPGN)的疗效。

材料与方法

我们治疗了28例患者(17例男性和11例女性,年龄19 - 71岁),其经形态学证实为ANCA相关性新月体性RPGN(新月体中位数为79(63:88)%)。患者接受了皮质类固醇和细胞毒性药物治疗。

结果

22例(78%)患者在8 - 16周时出现治疗反应:8例患者实现完全缓解,14例患者部分缓解。部分缓解患者的肾功能未完全恢复(肌酐中位数为200(180;255)μmol/l),持续存在中度蛋白尿(中位数为0.7(0.6;1.3)g/天)且无微血尿。治疗成功的概率取决于肾小球硬化的严重程度,与球囊外反应的活性相关性较弱。严重肾衰竭并非治疗失败的绝对预测指标。

结论

在无晚期肾硬化的情况下,早期使用皮质类固醇联合细胞毒性药物治疗可使70 - 80%的ANCA相关性RPGN患者产生积极疗效。

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