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以急性肾损伤为表现的肾血管炎。

Renal vasculitis presenting with acute kidney injury.

作者信息

Villacorta Javier, Diaz-Crespo Francisco, Acevedo Mercedes, Cavero Teresa, Guerrero Carmen, Praga Manuel, Fernandez-Juarez Gema

机构信息

Nephrology Department, Hospital Universitario Fundacion, C/Budapest 1, Alcorcon, 28922, Comunidad De Madrid, Spain.

Pathology Department, Hospital Virgen de la Salud, Toledo, Spain.

出版信息

Rheumatol Int. 2017 Jun;37(6):1035-1041. doi: 10.1007/s00296-017-3697-2. Epub 2017 Mar 13.

Abstract

Renal failure secondary to ANCA-associated vasculitis represents a clinical and therapeutic challenge. In this study, we aimed to assess the treatment response rates and long-term outcomes of vasculitis patients presenting with renal failure. This retrospective study included 151 patients with renal vasculitis from three hospitals who underwent a renal biopsy between 1997 and 2014. Patients with renal failure which required dialysis at the onset were compared to those presenting with more preserved renal function. The primary end point was treatment response and patient surivival. Patients with severe renal involvement had a lower response to treatment compared to those having preserved renal function (26.6 versus 93.4%; p < 0.001). Dialysis-dependent patients who received plasmapheresis in addition to immune suppressants associated a higher rate of renal recovery (41.6 versus 12.5%; p = 0.05). A higher incidence of severe infections was observed among patients with severe renal involvement (38.4 versus 18.1%, p = 0.01). The mortality rate was significantly higher among vasculitis patients presenting with renal failure (53.8 versus 22.2%, p = 0.001). Global survival at 1 and 5 years was 60 and 47% in patients requiring dialysis compared with 90 and 80% among those with more preserved renal function (p < 0.001). After multivariate adjustment, the need for dialysis remained as an independent predictor of death (HR 2.5; 95% CI 1.1-5.7; p = 0.03). The presence of severe renal dysfunction represents an independent risk factor for patient survival in renal vasculitis. Patients requiring dialysis associate a lower response rate to immunosuppressive therapy and a higher incidence of severe infections.

摘要

抗中性粒细胞胞浆抗体(ANCA)相关血管炎继发的肾衰竭是一项临床和治疗挑战。在本研究中,我们旨在评估出现肾衰竭的血管炎患者的治疗反应率和长期预后。这项回顾性研究纳入了1997年至2014年间在三家医院接受肾活检的151例肾血管炎患者。将起病时需要透析的肾衰竭患者与肾功能保留较好的患者进行比较。主要终点是治疗反应和患者生存率。与肾功能保留的患者相比,严重肾受累患者的治疗反应较低(26.6%对93.4%;p<0.001)。除免疫抑制剂外还接受血浆置换的依赖透析患者的肾功能恢复率较高(41.6%对12.5%;p=0.05)。严重肾受累患者中严重感染的发生率较高(38.4%对18.1%,p=0.01)。出现肾衰竭的血管炎患者的死亡率显著更高(53.8%对22.2%,p=0.001)。需要透析的患者1年和5年的总生存率分别为60%和47%,而肾功能保留较好的患者分别为90%和80%(p<0.001)。多变量调整后,透析需求仍然是死亡的独立预测因素(风险比2.5;95%置信区间1.1 - 5.7;p=0.03)。严重肾功能不全的存在是肾血管炎患者生存的独立危险因素。需要透析的患者对免疫抑制治疗的反应率较低,严重感染的发生率较高。

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