Management of childhood-onset systemic lupus erythematosus (cSLE) over the last two decades in Spain.

IF 2.8 3区 医学 Q1 PEDIATRICS
Alina Boteanu, Juan José Bethencourt, Joan Calzada-Hernández, Daniel Clemente, Juan Carlos Nieto-González, Covadonga López, Laura Luque, Inmaculada Calvo
{"title":"Management of childhood-onset systemic lupus erythematosus (cSLE) over the last two decades in Spain.","authors":"Alina Boteanu, Juan José Bethencourt, Joan Calzada-Hernández, Daniel Clemente, Juan Carlos Nieto-González, Covadonga López, Laura Luque, Inmaculada Calvo","doi":"10.1186/s12969-025-01113-3","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Childhood-onset systemic lupus erythematosus (cSLE) is a chronic multisystemic autoimmune disease with a more severe and life-threatening course than SLE in adults. Up to 50-80% of patients have renal or other major-organ involvement, such as in the neurological or nephrological systems, which results in significant morbidity and increased mortality.</p><p><strong>Main text: </strong>A search was conducted for lupus-related literature published by Spanish authors in PubMed, Science Direct, MEDES and SciELO databases. The search strategy was based on the keywords \"paediatric OR pediatric AND lupus AND Spain\" from 2005 to 2024. A manual search was also performed with the above keywords, including \"Clinical practice guidelines OR protocols OR recommendations\". Significant changes in the management of cSLE have occurred over the last 20 years in Spain. Since there is no consensus among experts at a national scale, the use of off-label drugs and the insufficiency of evidence-based recommendations have become widespread. Antimalarials and glucocorticoids have remained the cornerstone of treatment for several years. However, the side effects of the latter and their association with the accumulation of organ damage have led to the incorporation of immunosuppressants and biologic agents into clinical practice earlier in the course of the disease as glucocorticoids-sparing strategies. Recent advances in cSLE management include the application of the treat-to-target approach and the approval of intravenous belimumab for patients ≥ 5 years, while early results with subcutaneous belimumab in cSLE have as well demonstrated efficacy and safety.</p><p><strong>Conclusion: </strong>Despite the initiation of the Spanish Juvenile Systemic Lupus Erythematosus Registry in 2021, which aims to ascertain the outcomes of cSLE in Spanish patients, a consensus document for the management of these patients in Spain remains elusive. According to Treat to Target TaskGroup, treatment of cSLE should aim to ensure long-term survival and prevent organ damage. The use of glucocorticoid-sparing strategies, including biological agents such as belimumab, represents one of the main current challenges. Indeed, subcutaneous belimumab could be a promising tool in cSLE, as its administration in school-aged patients might reduce school absenteeism and lead to an improvement in the child's overall health.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"59"},"PeriodicalIF":2.8000,"publicationDate":"2025-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12107997/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Pediatric Rheumatology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1186/s12969-025-01113-3","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"PEDIATRICS","Score":null,"Total":0}
引用次数: 0

Abstract

Background: Childhood-onset systemic lupus erythematosus (cSLE) is a chronic multisystemic autoimmune disease with a more severe and life-threatening course than SLE in adults. Up to 50-80% of patients have renal or other major-organ involvement, such as in the neurological or nephrological systems, which results in significant morbidity and increased mortality.

Main text: A search was conducted for lupus-related literature published by Spanish authors in PubMed, Science Direct, MEDES and SciELO databases. The search strategy was based on the keywords "paediatric OR pediatric AND lupus AND Spain" from 2005 to 2024. A manual search was also performed with the above keywords, including "Clinical practice guidelines OR protocols OR recommendations". Significant changes in the management of cSLE have occurred over the last 20 years in Spain. Since there is no consensus among experts at a national scale, the use of off-label drugs and the insufficiency of evidence-based recommendations have become widespread. Antimalarials and glucocorticoids have remained the cornerstone of treatment for several years. However, the side effects of the latter and their association with the accumulation of organ damage have led to the incorporation of immunosuppressants and biologic agents into clinical practice earlier in the course of the disease as glucocorticoids-sparing strategies. Recent advances in cSLE management include the application of the treat-to-target approach and the approval of intravenous belimumab for patients ≥ 5 years, while early results with subcutaneous belimumab in cSLE have as well demonstrated efficacy and safety.

Conclusion: Despite the initiation of the Spanish Juvenile Systemic Lupus Erythematosus Registry in 2021, which aims to ascertain the outcomes of cSLE in Spanish patients, a consensus document for the management of these patients in Spain remains elusive. According to Treat to Target TaskGroup, treatment of cSLE should aim to ensure long-term survival and prevent organ damage. The use of glucocorticoid-sparing strategies, including biological agents such as belimumab, represents one of the main current challenges. Indeed, subcutaneous belimumab could be a promising tool in cSLE, as its administration in school-aged patients might reduce school absenteeism and lead to an improvement in the child's overall health.

管理儿童期发病系统性红斑狼疮(cSLE)在过去二十年在西班牙。
背景:儿童期全身性红斑狼疮(cSLE)是一种慢性多系统自身免疫性疾病,其病程比成人SLE更为严重和危及生命。高达50-80%的患者有肾脏或其他主要器官受累,如神经系统或肾脏系统,这导致显著的发病率和死亡率增加。全文:检索PubMed、Science Direct、MEDES和SciELO数据库中西班牙作者发表的狼疮相关文献。2005年至2024年的搜索策略是基于关键词“儿科或儿科和狼疮和西班牙”。手动搜索上述关键词,包括“临床实践指南或方案或建议”。在过去的20年里,西班牙的cSLE管理发生了重大变化。由于在全国范围内专家之间没有达成共识,使用标签外药物和缺乏循证建议已变得普遍。多年来,抗疟药和糖皮质激素一直是治疗的基石。然而,后者的副作用及其与器官损伤积累的关联导致免疫抑制剂和生物制剂作为糖皮质激素节约策略在疾病过程的早期纳入临床实践。cSLE治疗的最新进展包括治疗到靶点方法的应用和5年以上患者静脉注射贝利姆单抗的批准,而皮下贝利姆单抗治疗cSLE的早期结果也证明了有效性和安全性。结论:尽管西班牙青少年系统性红斑狼疮登记处于2021年启动,旨在确定西班牙患者的cSLE结局,但西班牙对这些患者的管理仍难以达成共识。根据治疗目标任务组,治疗cSLE应以确保长期生存和防止器官损害为目标。使用糖皮质激素保留策略,包括生物制剂如贝利单抗,是当前的主要挑战之一。事实上,皮下贝利姆单抗可能是一种很有前途的治疗cSLE的工具,因为在学龄患者中使用贝利姆单抗可能会减少缺勤,并改善儿童的整体健康状况。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
求助全文
约1分钟内获得全文 求助全文
来源期刊
Pediatric Rheumatology
Pediatric Rheumatology PEDIATRICS-RHEUMATOLOGY
CiteScore
4.10
自引率
8.00%
发文量
95
审稿时长
>12 weeks
期刊介绍: Pediatric Rheumatology is an open access, peer-reviewed, online journal encompassing all aspects of clinical and basic research related to pediatric rheumatology and allied subjects. The journal’s scope of diseases and syndromes include musculoskeletal pain syndromes, rheumatic fever and post-streptococcal syndromes, juvenile idiopathic arthritis, systemic lupus erythematosus, juvenile dermatomyositis, local and systemic scleroderma, Kawasaki disease, Henoch-Schonlein purpura and other vasculitides, sarcoidosis, inherited musculoskeletal syndromes, autoinflammatory syndromes, and others.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信