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狼疮性肾炎伴肾活检显示血栓性微血管病的临床转归和临床病理相关性。

Clinical Outcomes and Clinico-pathological Correlations in Lupus Nephritis with Kidney Biopsy Showing Thrombotic Microangiopathy.

机构信息

From the Division of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; Division of Nephrology, Department of Medicine, Queen Mary Hospital, University of Hong Kong; Department of Pathology, Queen Mary Hospital, Hong Kong.

C. Li, MD, Division of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College; D.Y. Yap, MBBS, MD, Division of Nephrology, Department of Medicine, Queen Mary Hospital, University of Hong Kong; G. Chan, MBBS, Department of Pathology, Queen Mary Hospital; Y.B. Wen, MMed, Division of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College; H. Li, MD, Division of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College; C. Tang, BSc, MSc, Division of Nephrology, Department of Medicine, Queen Mary Hospital, University of Hong Kong; X.M. Li, MD, PhD, Division of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College; X.W. Li, MMed, Division of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College; T.M. Chan, MBBS, MD, Division of Nephrology, Department of Medicine, Queen Mary Hospital, University of Hong Kong. CL and DYY contributed equally to this work.

出版信息

J Rheumatol. 2019 Nov;46(11):1478-1484. doi: 10.3899/jrheum.180773. Epub 2019 Mar 15.

Abstract

OBJECTIVE

Renal thrombotic microangiopathy (TMA) is an uncommon pathological finding in lupus nephritis (LN), and its clinical significance remains to be defined.

METHODS

Twenty-four patients with lupus nephritis (LN) and renal TMA were selected from a retrospective review of 677 biopsy-proven LN patients, and compared with 48 LN controls without TMA (1:2 ratio) matched according to demographics and treatments.

RESULTS

Renal TMA was noted in 3.5% of kidney biopsies of LN. TMA was associated with a higher prevalence of anti-Ro (45.8% vs 18.8%; p = 0.016), higher Systemic Lupus Erythematosus Disease Activity Index scores (21.4 ± 8.5 vs 10.8 ± 2.3; p < 0.001), lower estimated glomerular filtration rate (eGFR; 16.8 ± 11.7 ml/min vs 77.8 ± 28.6 ml/min; p < 0.001), and a higher percentage of patients who required dialysis (37.5% vs 2.1%; p < 0.001) at the time of kidney biopsy. Activity and chronicity indices [median (range)] were higher in the TMA group [11 (2-19) and 3 (1-8), respectively, compared with 7 (0-15) and 1 (0-3) in controls; p = 0.004 and p < 0.001; respectively]. Patients with TMA showed inferior 5-year renal survival and higher incidence of chronic kidney disease at last followup (70% and 66.6%, respectively, compared with 95% and 29.2% in controls; p = 0.023 and 0.002, respectively). The TMA group also showed lower median eGFR compared with controls [50.1 (IQR 7-132) ml/min vs 85.0 (IQR 12-147) ml/min; p = 0.003]. Five-year patient survival rate was similar between the 2 groups (87% and 98% in TMA and control group, respectively; p = 0.127).

CONCLUSION

TMA in kidney biopsy was associated with more severe clinical and histological activity, and significantly inferior longterm renal outcome in LN.

摘要

目的

血栓性微血管病(TMA)是狼疮性肾炎(LN)中一种罕见的病理发现,其临床意义仍有待确定。

方法

从 677 例经活检证实的 LN 患者的回顾性研究中选择 24 例 LN 合并肾 TMA 患者,并与 48 例无 TMA 的 LN 对照(1:2 比例)进行比较,这些对照患者按照人口统计学和治疗进行匹配。

结果

LN 肾活检中 TMA 的发生率为 3.5%。TMA 与更高的抗 Ro 抗体阳性率(45.8%比 18.8%;p = 0.016)、更高的系统性红斑狼疮疾病活动指数评分(21.4 ± 8.5 比 10.8 ± 2.3;p < 0.001)、更低的估算肾小球滤过率(eGFR;16.8 ± 11.7 ml/min 比 77.8 ± 28.6 ml/min;p < 0.001)和更高的需要透析的患者比例(37.5%比 2.1%;p < 0.001)相关。TMA 组的活动和慢性指数[中位数(范围)]更高[分别为 11(2-19)和 3(1-8),而对照组为 7(0-15)和 1(0-3);p = 0.004 和 p < 0.001]。TMA 组的 5 年肾脏存活率较低,在最后一次随访时发生慢性肾脏病的发生率较高(分别为 70%和 66.6%,而对照组分别为 95%和 29.2%;p = 0.023 和 0.002)。与对照组相比,TMA 组的中位 eGFR 也较低[50.1(IQR 7-132)ml/min 比 85.0(IQR 12-147)ml/min;p = 0.003]。两组的 5 年患者生存率相似(分别为 TMA 组 87%和对照组 98%;p = 0.127)。

结论

肾活检中的 TMA 与更严重的临床和组织学活动相关,并显著降低 LN 的长期肾脏预后。

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