Division of Rheumatology, Children's National Hospital, 111 Michigan Ave NW, Washington, DC, 20010, USA.
Department of Pediatrics, Johns Hopkins University, Baltimore, MD, USA.
Clin Rheumatol. 2022 Aug;41(8):2375-2381. doi: 10.1007/s10067-022-06146-7. Epub 2022 Mar 28.
Demographics, clinical features, and biomarkers do not consistently anticipate risk of end-stage renal disease (ESRD) in juvenile lupus nephritis (LN). Here, the existence of autoantibody clusters predictive of ESRD was explored in a cohort of biopsy-proven juvenile LN.
A retrospective chart review was performed of patients with juvenile systemic lupus erythematosus (jSLE) and biopsy-confirmed LN. Primary endpoints were ESRD and mortality. Patients were included for K-medians cluster analysis if they had a complete autoantibody profile, which included ANA titer, anti-dsDNA, anti-Smith, anti-RNP, anti-Ro/SSA, anti-La/SSB. Chi-square test was used for categorical variables and one-way ANOVA for continuous measures. Significance was p<0.05.
Fifty-three met inclusion criteria, of which 45 were female and 37 were black. Over 80% developed LN within one year of jSLE onset and more than half (n=29) had LN at diagnosis of jSLE. Six developed ESRD. No mortalities were reported. Forty-six had a complete autoantibody profile, including four with ESRD. Three clusters were identified. Group 1 (n=8) was defined by anti-dsDNA; group 2 (n=28) by high-titer ANA (>1:1280), anti-Smith, anti-RNP, and anti-Ro/SSA; and group 3 (n=10) by anti-dsDNA and anti-Ro/SSA. There was no difference between the groups in demographics, jSLE manifestations, or markers of renal function. One in group 2 and three in group 3 developed ESRD. Those in group 3 were younger at diagnosis of LN (p=0.084) and had the highest frequency of ESRD (p=0.025).
Cluster analysis revealed the highest frequency of ESRD in the group with LN defined by anti-Ro/SSA and anti-dsDNA co-positivity. Key Points • Lupus nephritis commonly is present at diagnosis of juvenile systemic lupus erythematosus or develops within the first year. • End-stage renal disease was more frequent in the cluster defined by anti-dsDNA and anti-Ro/SSA co-positivity; patients with this profile may benefit from more aggressive immunosuppression.
人口统计学、临床特征和生物标志物不能一致地预测青少年狼疮性肾炎（LN）的终末期肾病（ESRD）风险。在这里，在一组经活检证实的青少年 LN 中，探索了预测 ESRD 的自身抗体簇的存在。
对患有青少年系统性红斑狼疮（jSLE）和经活检证实的 LN 的患者进行回顾性图表审查。主要终点是 ESRD 和死亡率。如果患者具有完整的自身抗体谱，包括抗核抗体滴度、抗 dsDNA、抗 Smith、抗 RNP、抗 Ro/SSA、抗 La/SSB，则将其纳入 K-均值聚类分析。卡方检验用于分类变量，单向方差分析用于连续测量。显著性为 p<0.05。
53 名符合纳入标准，其中 45 名女性，37 名黑人。超过 80%的患者在 jSLE 发病一年内发生 LN，超过一半（n=29）在 jSLE 诊断时即发生 LN。有 6 人发展为 ESRD。没有报告死亡。46 人有完整的自身抗体谱，其中 4 人发生 ESRD。确定了 3 个聚类。第 1 组（n=8）由抗 dsDNA 定义；第 2 组（n=28）由高滴度抗核抗体（>1:1280）、抗 Smith、抗 RNP 和抗 Ro/SSA 定义；第 3 组（n=10）由抗 dsDNA 和抗 Ro/SSA 定义。各组在人口统计学、jSLE 表现或肾功能标志物方面无差异。第 2 组中有 1 人，第 3 组中有 3 人发展为 ESRD。第 3 组患者在 LN 诊断时更年轻（p=0.084），ESRD 发生率最高（p=0.025）。
聚类分析显示，由抗 Ro/SSA 和抗 dsDNA 同时阳性定义的 LN 组中 ESRD 发生率最高。关键点•狼疮性肾炎通常在青少年系统性红斑狼疮诊断时存在或在发病的第一年发生。•在由抗 dsDNA 和抗 Ro/SSA 同时阳性定义的聚类中，ESRD 更为常见；具有这种特征的患者可能受益于更积极的免疫抑制治疗。
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