Paediatric Nephrology Centre, Hong Kong Children's Hospital, Hong Kong.
Department of Paediatrics and Adolescent Medicine, University of Hong Kong, Hong Kong.
Rheumatology (Oxford). 2024 Apr 2;63(4):953-961. doi: 10.1093/rheumatology/kead447.
There is little data on renal relapse in childhood-onset LN (cLN). We investigate the incidence, predictive factors and outcomes related to renal relapse.
We conducted a retrospective cohort study of all cLN diagnosed at ≤18 years between 2001-2021 to investigate the incidence and outcomes related to renal relapse.
Ninety-five Chinese cLN patients (91% proliferative LN) were included. Induction immunosuppression was prednisolone and CYC [n = 36 (38%)] or MMF [n = 33 (35%)]. Maintenance immunosuppression was prednisolone and MMF [n = 53 (54%)] or AZA [n = 29 (31%)]. The rates of complete remission/partial remission (CR/PR) at 12 months were 78.9%/7.4%. Seventy renal relapses occurred in 39 patients over a follow-up of 10.2 years (s.d. 5.9) (0.07 episode/patient-year). Relapse-free survival was 94.7, 86.0, 80.1, 71.2, 68.3, 50.3 and 44.5% at 1, 2, 3, 4, 5, 10 and 20 years, respectively. Multivariate analysis showed that LN diagnosis <13.1 years [adjusted hazard ratio (HRadj) 2.59 995% CI 1.27, 5.29), P = 0.01], AZA maintenance [HRadj 2.20 (95% CI 1.01, 4.79), P = 0.05], PR [HRadj 3.9 (95% CI 1.03, 9.19), P = 0.01] and non-remission [HRadj 3.08 (95% CI 1.35, 11.3), P = 0.04] at 12 months were predictive of renal relapse. Renal relapse was significantly associated with advanced chronic kidney disease (stages 3-5) and end-stage kidney disease (17.9% vs 1.8%, P < 0.01). Furthermore, patients with renal relapse showed an increased incidence of infections (30.8% vs 10.7%, P = 0.02), osteopenia (38.5% vs 17.9%, P = 0.04) and hypertension (30.8% vs 7.1%, P < 0.01).
Renal relapse is common among cLN, especially among young patients, and is associated with an increased incidence of morbidity and mortality. Attaining CR and the use of MMF appear to decrease the incidence of renal relapse.
儿童期起病的狼疮性肾炎（cLN）的肾脏复发数据较少。我们调查了与肾脏复发相关的发生率、预测因素和结局。
我们对 2001 年至 2021 年间≤18 岁确诊的所有 cLN 患者进行了回顾性队列研究，以调查与肾脏复发相关的发生率和结局。
共纳入 95 例中国 cLN 患者（91%为增殖性 LN）。诱导免疫抑制方案为泼尼松龙和环磷酰胺[n=36（38%）]或霉酚酸酯[ n=33（35%）]。维持免疫抑制方案为泼尼松龙和霉酚酸酯[ n=53（54%）]或吗替麦考酚酯[ n=29（31%）]。12 个月时完全缓解/部分缓解（CR/PR）的比例为 78.9%/7.4%。在 10.2 年（标准差 5.9）的随访期间，39 例患者中有 70 例发生肾脏复发（0.07 例/患者年）。无复发生存率分别为 1 年时 94.7%、2 年时 86.0%、3 年时 80.1%、4 年时 71.2%、5 年时 68.3%、10 年时 50.3%和 20 年时 44.5%。多变量分析显示，LN 诊断年龄<13.1 岁[校正风险比（HRadj）2.59，995%置信区间（CI）1.27，5.29），P=0.01]、AZA 维持治疗[HRadj 2.20（95% CI 1.01，4.79），P=0.05]、PR[HRadj 3.9（95% CI 1.03，9.19），P=0.01]和 12 个月时未缓解[HRadj 3.08（95% CI 1.35，11.3），P=0.04]与肾脏复发相关。肾脏复发与慢性肾脏病晚期（3-5 期）和终末期肾病显著相关（17.9%vs1.8%，P<0.01）。此外，发生肾脏复发的患者感染（30.8%vs10.7%，P=0.02）、骨质疏松症（38.5%vs17.9%，P=0.04）和高血压（30.8%vs7.1%，P<0.01）的发生率更高。
cLN 患者肾脏复发较为常见，尤其是年轻患者，与发病率和死亡率的增加有关。达到 CR 和使用 MMF 似乎可以降低肾脏复发的发生率。
