NEUROPSYCHIATRIC SLE (NPSLE) IN CHILDREN AND ADOLESCENTS WITH LUPUS NEPHRITIS

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E-Poster

Abstract Title

First Name *

Eugene Yu Hin

Last Name *

Chan

Co-author 1

Lok Hei Matthew Wong matthew1032001@gmail.com Hong Kong Children’s Hospital Paediatric Nephrology Centre Hong Kong

Co-author 2

Ka Man Yip kmanyip@hku.hk The University of Hong Kong Department of Paediatrics and Adolescent Medicine Hong Kong

Co-author 3

Wilfred Hing Sang Wong whswong@hku.hk The University of Hong Kong Department of Paediatrics and Adolescent Medicine Hong Kong

Co-author 4

Alison Lap Tak Ma malta@ha.org.hk Hong Kong Children’s Hospital Paediatric Nephrology Centre Hong Kong

Co-author 5

Co-author 6

Co-author 7

Co-author 8

Co-author 9

Co-author 10

Co-author 11

Co-author 12

Co-author 13

Co-author 14

Co-author 15

Introduction

Data on neuropsychiatric manifestations in childhood-onset lupus nephritis (cLN) are scarce.

Methods

We conducted a retrospective cohort study of biopsy-proven cLN diagnosed from 2000 to 2021 to investigate the epidemiology, clinical characteristics, associated risk factors and outcomes of neuropsychiatric systemic lupus erythematosus (NPSLE) in the presence of cLN. NPSLE was defined according to the 1999 American College of Rheumatology nomenclature with the exclusion of those presenting with isolated headaches, anxiety or mood disorder.

Results

95 Chinese children with biopsy-proven cLN were included in the analysis. Of these patients, a total of 31 NPSLE events were reported in 11 patients (12%), with 1 patient developed NPSLE before the onset of cLN, 4 and 6 patients developed NPSLE concurrently with and after the onset of cLN, respectively. The most frequent NPSLE event was seizure (n=12, 39%). Estimated glomerular filtration rate <30ml/min/1.73m2 at diagnosis of cLN (adjusted odd ratio 6.73, 95% CI 1.29-35.11) and higher maximal proteinuria during the observation period (adjusted odd ratio 1.07, 95% CI 1-1.13) were predictive of NPSLE upon multivariable analysis.

Compared to children with cLN who did not develop NPSLE, significantly more children who developed subsequent NPSLE flare following initial kidney involvement had a history of medication non-adherence (100% vs 25%, p<0.001), higher degree of proteinuria at the diagnosis of cLN (urine protein/creatinine ratio, 5.74 vs 2.35mg/mg, p =0.04) and during the entire observation period (urine protein/creatinine ratio, 13.2 vs 3.3mg/mg, p = 0.004).

Patients with NPSLE had a significantly lower complete remission rates for cLN at 6- and 12-month post-induction (27.3% vs 70.2%, p = 0.014 ; 45.5% vs 83.3%, p =0.01, respectively). Kaplan-Meier analysis showed that patients with NPSLE had worse kidney (Figure 1) and patient survivals (Figure 2) (log-rank test, p <0.001, 0.0014 respectively) than those without NPSLE.

Figure 1: Kaplan Meier curve on renal survival in cLN children with and without NPSLE

Conclusions

Worse kidney and patient survivals are observed in cLN patients with NPSLE. Severe LN manifestation and medication non-adherence are associated with the development of NPSLE.

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