Lama Madhurima Veronica, Deepthi Bobbity, Krishnasamy Sudarsan, Ganesh Rajesh Nachiappa, Srinivas Bheemanathi Hanuman, Rajappa Medha, Krishnamurthy Sriram
Pediatric Nephrology Services, Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, 605006, India.
Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, 605006, India.
Pediatr Nephrol. 2025 Feb 1. doi: 10.1007/s00467-025-06666-9.
Limited data exists on clinical predictors of kidney outcomes in children with lupus nephritis (LN).
Children aged below 18 years with biopsy-proven LN followed from January 2010 to February 2024 in a tertiary-care center were enrolled in order to characterize their clinical presentations, flares, infections, histology and determine Major Adverse Kidney Events (MAKE) (defined as eGFR < 60 mL/min/1.73 m and/or death). Data was analysed to identify predictive factors of adverse outcomes.
Seventy-five children (20% boys) with median (IQR) age at diagnosis 11.2 (9,13) years, were studied. Clinical presentations were nephritic syndrome, mixed nephritic-nephrotic features, rapidly progressive glomerulonephritis (RPGN), and nephrotic syndrome in 24 (32%), 15 (20%), 11 (14.7%) and 5 (6.7%) patients, respectively. Proliferative LN was the major (70.6%) histological subtype. In total, 75 kidney flares (21.3% nephritic) with incidence rate (IR) of 0.48 flares per person-year were noted in 31 (41.3%) children. Infections occurred in 32 (42.6%), with IR of 0.58 episodes per person-year. Bacterial pneumonia 14 (22.9%), sepsis 10 (16.3%) and tropical infections 6 (9.8%) were most common. At median last follow-up of 2.3 (1.3, 5.6) years with 85.4% kidney-survival rate, 41 (54.6%), and 21 (28%) were in complete-response (CR), and partial-response (PR), respectively. Proliferative LN and those in PR or NR were at significantly higher risk of kidney flares and infections, regardless of initial induction therapy. RPGN at presentation, non-responders at 6 months and severe kidney flare ever predicted MAKE in 11 (14.6%) children.
Multiple kidney flares and infections constitute a significant morbidity in LN. RPGN, non-responders and severe kidney flare predict adverse kidney outcomes.
关于狼疮性肾炎（LN）患儿肾脏预后的临床预测因素的数据有限。
纳入2010年1月至2024年2月在一家三级医疗中心随访的18岁以下经活检证实为LN的儿童，以描述其临床表现、病情复发、感染、组织学特征，并确定主要不良肾脏事件（MAKE）（定义为估算肾小球滤过率[eGFR]<60 mL/min/1.73 m²和/或死亡）。对数据进行分析以确定不良预后的预测因素。
研究了75名儿童（20%为男孩），诊断时的中位（四分位间距）年龄为11.2（9，13）岁。临床表现分别为肾病综合征、混合性肾炎-肾病特征、快速进展性肾小球肾炎（RPGN）和肾病综合征的患儿有24例（32%）、15例（20%）、11例（14.7%）和5例（6.7%）。增殖性LN是主要的（70.6%）组织学亚型。总共31名（41.3%）儿童出现75次肾脏病情复发（21.3%为肾炎性），发病率为每人年0.48次复发。32例（42.6%）发生感染，发病率为每人年0.58次。最常见的是细菌性肺炎14例（22.9%）、败血症10例（16.3%）和热带感染6例（9.8%）。在中位末次随访2.3（1.3，5.6）年时，肾脏存活率为85. %，分别有41例（54.6%）和21例（28%）处于完全缓解（CR）和部分缓解（PR）状态。无论初始诱导治疗如何，增殖性LN以及处于PR或未缓解（NR）状态的患儿发生肾脏病情复发和感染的风险显著更高。就诊时为RPGN、6个月时无反应者以及曾出现严重肾脏病情复发可预测11例（14.6%）儿童发生MAKE。
多次肾脏病情复发和感染在LN中构成显著的发病率。RPGN、无反应者和严重肾脏病情复发可预测不良肾脏预后。
