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在 presentation 时需要透析的重症 ANCA 相关性血管炎肾炎患者的预后预测因素:单中心 89 例研究。

Predictors for outcomes in patients with severe ANCA-associated glomerulonephritis who were dialysis-dependent at presentation: a study of 89 cases in a single Chinese center.

机构信息

Renal Division, Department of Medicine, Peking University First Hospital, Beijing, China.

出版信息

Semin Arthritis Rheum. 2013 Apr;42(5):515-21. doi: 10.1016/j.semarthrit.2012.09.005. Epub 2013 Jan 16.

Abstract

OBJECTIVE

Anti-neutrophilcytoplasmic autoantibody (ANCA)-associated vasculitis may cause rapid deterioration of renal function, resulting in high prevalence of end-stage renal disease and mortality. The current study investigated factors associated with restoration of renal function and early mortality in patients with severe ANCA-associated glomerulonephritis, i.e. dialysis-dependent at presentation, in a single Chinese cohort.

METHODS

Eighty-nine Chinese patients with ANCA-associated glomerulonephritis who were on dialysis at the time of diagnosis were included in this study. All these patients received immunosuppressive therapy plus intravenous methylprednisolone, plasma exchange, or both. The predictive value of the clinical and histological parameters for renal and patient outcome was analyzed.

RESULT

On the sixth month, 25 (28.1%) patients achieved dialysis independence, 45 (50.6%) patients progressed to end stage renal disease, and 19 (21.3%) patients died. Nine out of the 19 deaths were therapy-related. Factors independently associated with renal function restoration were percentages of normal glomeruli (P<0.05), extent of tubular atrophy (P<0.05) and extent of interstitial fibrosis (P<0.05) in the renal specimens. Age and pulmonary hemorrhage were independently associated with all-cause death (P=0.003 and P=0.007, respectively) and therapy-related death (P=0.037 and P=0.043, respectively).

CONCLUSIONS

Among patients with severe ANCA-associated glomerulonephritis who were dialysis-dependent at presentation, those with a higher percentage of normal glomeruli and less extent of tubular atrophy/interstitial fibrosis have more chance of restoration of renal function. Increased risk for all-cause death and therapy-related death appears to be older age and pulmonary hemorrhage.

摘要

目的

抗中性粒细胞胞浆抗体(ANCA)相关性血管炎可导致肾功能迅速恶化,从而导致终末期肾病和死亡率居高不下。本研究调查了在中国单一队列中,表现为透析依赖的严重 ANCA 相关性肾小球肾炎患者肾功能恢复和早期死亡率的相关因素。

方法

本研究纳入了 89 名在诊断时即接受透析的中国 ANCA 相关性肾小球肾炎患者。所有患者均接受免疫抑制治疗加静脉甲基强的松龙、血浆置换或两者联合治疗。分析了临床和组织学参数对肾脏和患者结局的预测价值。

结果

在第六个月时,25 名(28.1%)患者实现了透析独立,45 名(50.6%)患者进展为终末期肾病,19 名(21.3%)患者死亡。19 例死亡中有 9 例与治疗相关。与肾功能恢复相关的独立因素是肾组织中正常肾小球的比例(P<0.05)、肾小管萎缩的程度(P<0.05)和间质纤维化的程度(P<0.05)。年龄和肺出血与全因死亡(P=0.003 和 P=0.007)和治疗相关死亡(P=0.037 和 P=0.043)独立相关。

结论

在表现为透析依赖的严重 ANCA 相关性肾小球肾炎患者中,正常肾小球比例较高且肾小管萎缩/间质纤维化程度较低的患者更有可能恢复肾功能。全因死亡和治疗相关死亡的风险似乎与年龄较大和肺出血有关。

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