Data_Sheet_1_Pediatric Systemic Lupus Erythematosus: Learning From Longer Follow Up to Adulthood.PDF

Background: Pediatric systemic lupus erythematosus (pSLE) is a rare condition, representing approximately 10% of SLE cases. The aim of this study was to identify variables to improve the diagnostic awareness and management of pSLE patients.
Methods: This retrospective study included 25 patients diagnosed with pSLE and followed at the University of Pisa. We collected data about clinical profile at disease onset and during a long-term follow-up, including disease activity, organ damage development, and treatments received.
Results: The mean patient age at disease onset was 14.6 ± 1.6 years, and the mean follow-up period was 14.17 ± 8.04 years. The most common initial manifestations were arthritis, malar rash, and cytopenias. The median time to diagnosis since the first symptoms was 6 months, and was significantly longer in patients with hematological onset (54 months). During follow-up, the number of patients with renal involvement showed a significant increase, from 36% at diagnosis to 72.2% after 10 years of disease evolution. Patients who developed chronic organ damage maintained a higher time-averaged disease activity during follow-up and received a significantly higher dose of corticosteroids.
Conclusion: Patients with immune cytopenia represent a group deserving strict clinical follow-up for the risk of evolution to SLE. Intense surveillance of renal function, early treatment and steroid-sparing strategies should be strongly considered in the management of pSLE patients.

背景：儿童系统性红斑狼疮（pediatric systemic lupus erythematosus, pSLE）是一种罕见疾病，约占系统性红斑狼疮（systemic lupus erythematosus, SLE）总病例的10%。本研究旨在识别相关变量，以提升pSLE患者的诊断认知与临床管理水平。
方法：本回顾性研究纳入了25例于比萨大学（University of Pisa）确诊并接受随访的pSLE患者。我们收集了患者疾病发作时及长期随访期间的临床特征数据，涵盖疾病活动度、器官损害发生情况及所接受的治疗方案。
结果：患者发病时的平均年龄为14.6±1.6岁，平均随访时长为14.17±8.04年。最常见的初始临床表现为关节炎、颧部红斑及血细胞减少。从首次出现症状到确诊的中位时间为6个月，其中以血液系统为首发表现的患者确诊延迟时间显著更长（54个月）。随访期间，出现肾脏受累的患者比例从确诊时的36%显著升至病程10年后的72.2%。发生慢性器官损害的患者，其随访期间的时间平均疾病活动度更高，且接受的糖皮质激素剂量显著更高。
结论：以免疫性血细胞减少为首发表现的患者，存在进展为SLE的风险，属于需严格临床随访的人群。在pSLE患者的临床管理中，应强化肾功能监测、早期干预及采用糖皮质激素节约策略。