Background Awareness of paediatric-specific predictors of damage in Childhood-lupus is needed to inform mitigation measures. Objectives To ascertain how clinical and demographic variables correlate with damage accrual and identify predictors of damage. Methods Analysis included UK JSLE Cohort Study participants. Univariable and multivariable Prentice-Williams-Peterson models investigated how demographic and clinical factors influenced hazards of new damage. Analyses were performed across the entire cohort, in patients with minimal disease activity marked by a time-adjusted average SLEDAI-2K score (AMS)≤2, low activity (AMS ≤ 4), moderate-high activity (AMS > 4) and those with no corticosteroids. Results Within the entire cohort (n = 430), factors associated with damage included: any methylprednisolone (Hazard Ratio, HR 2.20, [CI 1.33–3.62]), time-adjusted mean Physicians Global Assessment (PGA) (HR 2.87, [CI 1.48–5.56]) and AMS score (HR 1.13, [CI 1.03–1.24], all p< 0.05). Within the low activity subgroup, any methylprednisolone (HR 2.61, [CI 1.04–6.53]) and time-adjusted mean PGA (HR 3.41, [CI 1.52–7.76]) were associated with damage (both p< 0.05). Within the moderate-high activity subgroup, any methylprednisolone (HR 2.29, [CI 1.31–4.00]), time-adjusted mean PGA (HR 2.66, [CI 1.20–5.87]) and AMS score (HR 1.15, [CI 1.03–1.29]), were predictive of damage (all p< 0.05). Baseline organ damage was predictive of subsequent damage accrual in the minimal activity (HR 1.33, CI [1.78–8.08]) and no corticosteroids subgroups (HR 3.64, CI [1.83–7.24], both p< 0.005). Conclusion Disease activity levels (AMS/PGA) and proxy indicators (methylprednisolone exposure, baseline damage) were found to be key predictors of damage accrual. This highlights the importance of practical strategies, to reduce disease activity and long-term treatment toxicity, such as treat-to-target.

中文翻译：

---

导致儿童狼疮器官损伤的原因：皮质类固醇的使用和疾病活动


背景 需要了解儿童狼疮损伤的儿科特异性预测因子，以便为缓解措施提供信息。目的 确定临床和人口统计学变量如何与损伤累积相关，并确定损伤的预测因子。方法 分析包括英国 JSLE 队列研究参与者。单变量和多变量 Prentice-Williams-Peterson 模型研究了人口统计学和临床因素如何影响新损伤的危害。对整个队列进行了分析，在以时间调整平均 SLEDAI-2K 评分 （AMS）≤2 、低活动度 （AMS ≤ 4）、中高活动度 （AMS > 4） 和无皮质类固醇的患者为特征的最小疾病活动度患者中进行分析。结果在整个队列 （n = 430） 中，与损伤相关的因素包括：任何甲泼尼龙（风险比，HR 2.20，[CI 1.33-3.62]）、时间调整平均医师整体评估 （PGA） （HR 2.87，[CI 1.48-5.56]）和 AMS 评分 （HR 1.13，[CI 1.03-1.24]，均 p< 0.05）。在低活性亚组中，任何甲泼尼龙 （HR 2.61， [CI 1.04-6.53]） 和时间调整的平均 PGA （HR 3.41， [CI 1.52-7.76]） 都与损伤相关 （均 p< 0.05）。在中高活性亚组中，任何甲泼尼龙 （HR 2.29， [CI 1.31–4.00]）、时间调整后的平均 PGA （HR 2.66， [CI 1.20–5.87]）和 AMS 评分 （HR 1.15， [CI 1.03–1.29]），均可预测损伤 （均 p< 0.05）。基线器官损伤可预测最小活动组（HR 1.33，CI [1.78–8.08]）和无皮质类固醇亚组（HR 3.64，CI [1.83–7.24]，均p< 0.005）的后续损伤累积。结论 疾病活动水平 （AMS/PGA） 和代理指标 （甲泼尼龙暴露、基线损伤） 是损伤累积的关键预测因子。 这凸显了实用策略的重要性，以减少疾病活动和长期治疗毒性，例如靶向治疗。