伴有血栓性微血管病的增殖性狼疮性肾炎的结局；一项回顾性和前瞻性观察性单中心研究。

Outcome of proliferative lupus nephritis with thrombotic microangiopathy; An ambispective observational single-center study.

作者信息

Fayed Ahmed, Elgohary Rasmia, Shaker Amr Mohamed, Salem Karem Mohamed, Desouky Eman El, Maghraby Gehad Gamal

机构信息

Nephrology Unit, Internal Medicine Department, Kasr Alainy School of Medicine, Cairo University, Cairo, Egypt.

Rheumatology and Clinical Immunology Unit, Internal Medicine Department, Kasr Alainy School of Medicine, Cairo University, Cairo, Egypt.

出版信息

BMC Nephrol. 2025 May 13;26(1):233. doi: 10.1186/s12882-025-04154-8.

DOI:10.1186/s12882-025-04154-8

PMID:40361003

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12076891/

Abstract

BACKGROUND

Thrombotic microangiopathy (TMA) represents a broad spectrum of diseases. The combination of TMA with lupus nephritis (LN) is associated with worse renal outcomes and a higher mortality rate. To date, there is no agreement on the therapeutic strategies that should be offered to TMA-LN patients.

OBJECTIVE

In this study, we compared the long-term outcomes of plasma exchange (PLEX) and cyclophosphamide (CYC) in a TMA-LN cohort.

METHODS

100 TMA-LN patients who received an induction of steroids and either PLEX or CYC less than 3 months from the start of the study, were selected from the medical records of Kasr Alainy hospitals, Cairo University. The patients were monitored for hematological and renal response at 3, 6, and 12 months.

FINDINGS

In PLEX arm, the mean creatinine level was 1.4 ± 0.7 mg/dl at baseline, decreased to 1.1 ± 0.5 mg/dl after 3 months, and returned to 1.4 ± 1.4 mg/dl at 6 and 12 months (p = 0.748). Proteinuria levels significantly decreased from 2.9 ± 0.7 9 gm/24 hrs at baseline to 0.4 ± 0.5 9 gm/24 hrs after 12 months (p < 0.001). PLT significantly increased over time with a mean of 65.6 ± 19.0 (x10)/L at baseline, increasing to 235.9 ± 54.3 (x10)/L after 12 months (p < 0.001). In CYC arm, the mean creatinine level of 1.2 ± 0.6 mg/dl was maintained from baseline through 6 months but significantly increased at 12 months with a mean of 1.9 ± 2.2 mg/dl (p = 0.005). Proteinuria levels significantly decreased with means of 3.3 ± 0.6 gm/24 hrs at baseline to 0.7 ± 0.9 gm/24 hrs after 12 months (p < 0.001). The PLT increased from 49.5 ± 19.0 (x10)/L to 198.9 ± 71.5 (x10)/L after 12 months (p < 0.001). At 3- and 12-month intervals, PLEX achieved sustained lower proteinuria (p < 0.001 and p = 0.047, respectively), higher PLT (p < 0.001 and p = 0.005, respectively), and higher complement 4 (p = 0.001 and p < 0.001, respectively), compared to CYC.

CONCLUSION

Both groups demonstrated significant improvements in renal and hematological outcomes with better long-term renal outcomes in the PLEX arm and comparable improvements in the hematological measures in both groups.

摘要

背景

血栓性微血管病（TMA）代表了一系列广泛的疾病。TMA与狼疮性肾炎（LN）的合并与更差的肾脏结局和更高的死亡率相关。迄今为止，对于应提供给TMA-LN患者的治疗策略尚无共识。

目的

在本研究中，我们比较了血浆置换（PLEX）和环磷酰胺（CYC）在TMA-LN队列中的长期结局。

方法

从开罗大学Kasr Alainy医院的病历中选取100例TMA-LN患者，这些患者在研究开始后3个月内接受了类固醇诱导治疗，并接受了PLEX或CYC治疗。在3、6和12个月时对患者进行血液学和肾脏反应监测。

结果

在PLEX组中，基线时平均肌酐水平为1.4±0.7mg/dl，3个月后降至1.1±0.5mg/dl，6个月和12个月时恢复至1.4±1.4mg/dl（p = 0.748）。蛋白尿水平从基线时的2.9±0.79g/24小时显著降至12个月后的0.4±0.59g/24小时（p < 0.001）。血小板随时间显著增加，基线时平均为65.6±19.0（×10）/L，12个月后增至235.9±54.3（×10）/L（p < 0.001）。在CYC组中，平均肌酐水平从基线至6个月维持在1.2±0.6mg/dl，但在12个月时显著升高，平均为1.9±2.2mg/dl（p = 0.005）。蛋白尿水平从基线时的3.3±0.6g/24小时显著降至12个月后的0.7±0.9g/24小时（p < 0.001）。血小板在12个月后从49.5±19.0（×10）/L增至198.9±71.5（×10）/L（p < 0.001）。在3个月和12个月时，与CYC相比，PLEX实现了持续更低的蛋白尿（分别为p < 0.001和p = 0.047）、更高的血小板（分别为p < 0.001和p = 0.005）以及更高的补体4（分别为p = 0.001和p < 0.001）。