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.
J Rheumatol. 2019 Nov;46(11):1478-1484. doi: 10.3899/jrheum.180773. Epub 2019 Mar 15.
Renal thrombotic microangiopathy (TMA) is an uncommon pathological finding in lupus nephritis (LN), and its clinical significance remains to be defined.
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.
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).
TMA in kidney biopsy was associated with more severe clinical and histological activity, and significantly inferior longterm renal outcome in LN.
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。
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。
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)。
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。