背景:本研究旨在确定儿童期狼疮性肾炎(cLN)中肾血管病变(RVL)的临床意义。
方法:我们回顾性回顾了2004-2020年间所有经活检证实的cLN患儿,以评估肾活检中RVLs的患病率及其相关因素和长期结局。复合肾脏结局定义为晚期慢性肾脏疾病(CKD)3-5期,肾衰竭和死亡。
结果:分析了84例中国患者的107例活检。在19例患者中观察到RVL(22.6%),包括非炎性坏死性血管病变(NNV,n=6),血栓性微血管病(TMA,n=4),动脉硬化(AS,n=3),与AS并发NNV(n=4),并发NNV与TMA(n=1)和并发真性肾血管炎与AS(n=1)。RVL的存在与较低的估计肾小球滤过率(eGFR)(66.9±40.3vs.95.6±39.4ml/min/1.73m2,p=0.005),血红蛋白水平(9.1±1.9vs.10.4±1.9g/dL,p=0.008)和血小板计数(150.1±96.4vs.217.2±104.8×109/L,p=0.01)。LN类别和活动/慢性指数相似。RVL患者的综合肾脏结局较差,虽然没有达到统计学意义(对数秩检验,p=0.06)。NNV的存在与无复合肾脏结局的低生存率相关(对数秩检验,p=0.0018),与其他形式的RVL和没有RVL的RVL相比。单因素分析显示,NNV(HR7.08,95%CI1.67-30.03)可预测复合肾脏结局。
结论:RVL存在于五分之一的cLN患者中,并与严重表现相关。NNV与更坏的长期肾脏结果相关。RVL的常规评估是必要的,应纳入未来的分类标准。
BACKGROUND: This study aimed to determine the clinical significance of renal vascular lesions (RVLs) in childhood-onset lupus nephritis (cLN).
METHODS: We retrospectively reviewed all children with biopsy-proven cLN between 2004-2020 to evaluate the prevalence of RVLs on kidney biopsy and its associated factors and long-term outcomes. The composite kidney outcome was defined as advanced chronic kidney disease (CKD) stage 3-5, kidney failure and death.
RESULTS: 107 biopsies from 84 Chinese patients were analysed. RVLs were observed in 19 patients (22.6%), including non-inflammatory necrotizing vasculopathy (NNV, n = 6), thrombotic microangiopathy (TMA, n = 4), arterial sclerosis (AS, n = 3), concurrent NNV with AS (n = 4), concurrent NNV with TMA (n = 1) and concurrent true renal vasculitis with AS (n = 1). The presence of RVLs was associated with lower estimated glomerular filtration rate (eGFR) (66.9 ± 40.3 vs. 95.6 ± 39.4 ml/min/1.73m2, p = 0.005), haemoglobin level (9.1 ± 1.9 vs. 10.4 ± 1.9 g/dL, p = 0.008) and platelet count (150.1 ± 96.4 vs. 217.2 ± 104.8 × 109/L, p = 0.01). LN classes and activity/chronicity indices were similar. Patients with RVLs had poorer composite kidney outcomes, though not reaching statistical significance (log-rank test, p = 0.06). The presence of NNV was associated with inferior survival free from composite kidney outcome (log-rank test, p = 0.0018), compared to other forms of RVLs and those without RVLs. Univariate analysis revealed NNV (HR 7.08, 95% CI 1.67-30.03) was predictive of composite kidney outcome.
CONCLUSIONS: RVLs are present in one-fifth of cLN patients and are associated with severe presentation. NNV is associated with worse long-term kidney outcome. Routine evaluation of RVLs is warranted and should be incorporated into future classification criteria.