Division of Nephrology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Division of Nephrology, Department of Medicine, Emory University School of Medicine, Atlanta, GA, USA.
J Nephrol. 2021 Dec;34(6):1907-1913. doi: 10.1007/s40620-021-01010-4. Epub 2021 Mar 10.
Kidney involvement is common in patients with systemic lupus erythematosus (SLE). This study investigates the clinical and prognostic characteristics of thrombotic microangiopathy (TMA) compared to class IV lupus nephritis in SLE patients.
A retrospective review of patients who underwent kidney biopsy, with a primary diagnosis of SLE and TMA between June 2006 and September 2018 was conducted. Those patients were subsequently compared to patients with class IV lupus nephritis between January 2018 and December 2018. Demographics, laboratory, and serological data at the time of biopsy were abstracted.
Among 214 SLE patients records screened, 27 were included in the final analysis. Eight patients had lupus-related TMA without evidence of active lupus nephritis, while 19 patients had class IV lupus nephritis without evidence of TMA. TMA patients had significantly higher lactate dehydrogenase levels (718 ± 499 vs. 264 ± 107.7 U/L, p = 0.009), serum C3 (100.6 ± 39.3 vs. 65.8 ± 27 mg/dL, p = 0.049), white blood cell count (14743.8 ± 7933.3 vs. 5807.9 ± 2053.2 × 10E3/uL, p < 0.001), and total bilirubin (0.8 ± 0.5 vs. 0.3 ± 0.1 mg/dL, p = 0.007) in addition to significantly lower platelet counts (158.4 ± 88.6 vs. 240.3 ± 100.3 × 10E3/uL, p = 0.03), and haptoglobin (68.8 ± 116.1 vs. 166.8 ± 95.4 mg/dL, p = 0.03). After a median follow-up time of 53 weeks, 3 patients with TMA were dialysis-dependent (37.5%), compared with none in class IV lupus nephritis patients (p = 0.002).
TMA-associated SLE has worse prognosis compared to class IV lupus nephritis. An array of laboratory and pathological findings may be of value in discriminating between those two entities.
肾脏受累在系统性红斑狼疮（SLE）患者中很常见。本研究旨在比较血栓性微血管病（TMA）与狼疮性肾炎 IV 型（IV 型 LN）患者的临床和预后特征。
回顾性分析 2006 年 6 月至 2018 年 9 月期间接受肾活检且初诊为 SLE 合并 TMA 的患者，随后将这些患者与 2018 年 1 月至 2018 年 12 月期间接受肾活检且确诊为 IV 型 LN 的患者进行比较。记录患者的人口统计学、实验室和血清学数据。
在筛选的 214 例 SLE 患者记录中，最终有 27 例纳入最终分析。8 例患者存在狼疮相关性 TMA 但无活动性狼疮肾炎证据，19 例患者存在 IV 型 LN 但无 TMA 证据。TMA 患者的乳酸脱氢酶水平（718±499 比 264±107.7 U/L，p=0.009）、血清 C3（100.6±39.3 比 65.8±27 比 mg/dL，p=0.049）、白细胞计数（14743.8±7933.3 比 5807.9±2053.2×10E3/uL，p<0.001）和总胆红素（0.8±0.5 比 0.3±0.1 mg/dL，p=0.007）显著更高，血小板计数（158.4±88.6 比 240.3±100.3×10E3/uL，p=0.03）和结合珠蛋白（68.8±116.1 比 166.8±95.4 mg/dL，p=0.03）显著更低。中位随访时间为 53 周后，3 例 TMA 患者依赖透析（37.5%），而 IV 型 LN 患者无一例依赖透析（p=0.002）。
与 IV 型 LN 相比，TMA 相关的 SLE 预后更差。一系列实验室和病理发现可能有助于区分这两种疾病。
