High disease activity persists in adults with childhood-onset lupus.

IF 4.2 2区医学 Q1 RHEUMATOLOGY

Lupus Science & Medicine Pub Date : 2026-08-05 DOI:10.1136/lupus-2026-002138

Rachel Koelmeyer, Kate Gregory, Rangi Kandane-Rathnayake, Fiona Goldblatt, Sean O'Neill, Maureen Rischmueller, Mandana Nikpour, Geraldine Hassett, Pravin Hissaria, Dwarakanathan Ranganathan, Claire Barrett, Ashleigh Hennessey, Ted Tsai, Peter Gowdie, Eric F Morand, Alberta Hoi

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引用次数: 0

Abstract

Objective: Childhood-onset SLE (cSLE) is often described as more severe than adult-onset disease (aSLE) based largely on cross-sectional studies. We examined differences in disease characteristics, medication use and long-term outcomes between cSLE and patients with aSLE in adulthood using longitudinal data from a national cohort to address this knowledge gap.

Methods: A retrospective cohort study was conducted using the Australian Lupus Registry and Biobank. Data included demographics, disease duration, autoantibody profile, classification criteria, time-adjusted mean SLE Disease Activity Index 2000 (SLEDAI-2K AMS), SLE Damage Index (SDI) and SF36 health-related quality of life data. Key outcomes were AMS and time in the Lupus Low Disease Activity State (LLDAS) and damage accrual. Bivariate tests were used for group comparisons.

Results: Of 519 patients with SLE enrolled between 2011 and 2022 with ≥12 months of data available, 68 (13%) had cSLE. Median (IQR) age at enrolment was 39 (30-51) years; 88.1% female; majority were of white or Asian ethnicity. At baseline, more patients with cSLE had damage (SDI≥1) (53% vs 40%, p=0·05) and renal involvement (62% vs 37%, p<0·001). Over median (IQR) follow-up of 5 (2·6-9·3) years, patients with cSLE had higher disease activity (median (IQR) AMS 5·0 (3·0-6·4) cSLE vs 3·6 (1·9-5·1) aSLE, p<0.001), were more likely to be in High Disease Activity Status (SLEDAI-2K ≥10 ever) and were less likely to achieve LLDAS-50 (36% vs 51%, p=0·036). Flare rates, damage accrual and quality of life during follow-up were similar between groups.

Conclusions: Adults with cSLE entered adult follow-up with higher baseline damage and continued to experience a higher longitudinal disease activity burden than patients with aSLE. These findings highlight the importance of early recognition, consistent longitudinal monitoring and timely escalation of therapy during earlier years of disease to reduce long-term disease burden.

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成人儿童期狼疮患者疾病活动性高。

目的：主要基于横断面研究，儿童发病的SLE （cSLE）通常被描述为比成人发病的疾病（aSLE）更严重。我们使用来自国家队列的纵向数据来研究成人期cSLE和aSLE患者在疾病特征、药物使用和长期结局方面的差异，以解决这一知识差距。方法：使用澳大利亚狼疮登记处和生物库进行回顾性队列研究。数据包括人口统计学、病程、自身抗体谱、分类标准、时间调整后的SLE疾病活动指数2000 （SLEDAI-2K AMS）、SLE损害指数（SDI）和SF36健康相关生活质量数据。主要结局是AMS和狼疮低疾病活动状态（LLDAS）的时间和损伤累积。双变量检验用于组间比较。结果：在2011年至2022年期间入组的519例SLE患者中，有≥12个月的可用数据，68例（13%）患有SLE。入组时的中位年龄（IQR）为39岁（30-51岁）；88.1%的女性;大多数是白人或亚裔。在基线时，更多的cSLE患者有损害（SDI≥1）（53% vs 40%, p= 0.05）和肾脏受累（62% vs 37%）。结论：与aSLE患者相比，成人cSLE患者在进入成人随访时基线损害更高，并且继续经历更高的纵向疾病活动负担。这些发现强调了早期识别、持续的纵向监测和在疾病早期及时升级治疗以减少长期疾病负担的重要性。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Lupus Science & Medicine RHEUMATOLOGY-

CiteScore

5.30

自引率

7.70%

发文量

审稿时长

15 weeks

期刊介绍： Lupus Science & Medicine is a global, peer reviewed, open access online journal that provides a central point for publication of basic, clinical, translational, and epidemiological studies of all aspects of lupus and related diseases. It is the first lupus-specific open access journal in the world and was developed in response to the need for a barrier-free forum for publication of groundbreaking studies in lupus. The journal publishes research on lupus from fields including, but not limited to: rheumatology, dermatology, nephrology, immunology, pediatrics, cardiology, hepatology, pulmonology, obstetrics and gynecology, and psychiatry.