摘要
儿童系统性红斑狼疮(cSLE)临床表现多样,除部分患儿有发热、皮疹等典型表现外,因受累脏器不同,而使临床表现各异,其中肾脏是cSLE患儿最易受累器官,也是导致其死亡的主要原因之一。对cSLE的早期识别、评估疾病活动度、选择合适治疗方案,可有效保护该病患儿的肾功能,改善患儿预后是治疗关键。既往对狼疮性肾炎(LN)患儿多关注肾小球受累情况,而且以肾小球病理损害,作为LN临床分型依据,目前越来越多的研究提示,cSLE相关肾小管间质损伤(TIN),虽然无特异性临床表现,但是对LN患儿的治疗和改善患儿预后有显著影响。笔者拟就LN患儿的临床诊断及发病机制、cSLE TIN的临床意义及TIN的预测指标、LN患儿TIN的临床表现的最新研究现状进行阐述,旨在提高临床对LN患儿cSLE相关TIN的认识,进一步加强对儿童LN患儿的管理。
The clinical manifestations of childhood-onset systemic lupus erythematosus(cSLE)are diverse,except for some typical manifestations of fever and rash,and the clinical manifestations are different due to different organs involved,among which the kidney is the most susceptible organ with cSLE,and also one of the main causes of death.Early identification of cSLE,assessment of disease activity,and selection of appropriate treatment regimens can effectively protect the renal function of children with this disease,and improving the prognosis of children is the key to treatment.In the past,children with lupus nephritis(LN)paid more attention to glomerular involvement,and the pathological damage of the glomerulus was used as the basis for clinical classification of LN,and more and more studies suggest that cSLE-related tubulointerstitial injury(TIN)in children,although there is no specific clinical manifestation,but it has a significant impact on the treatment of children with LN and the improvement of their prognosis.The author intends to elaborate on the clinical diagnosis and pathogenesis of LN children,the clinical significance and predictive indicators of TIN in LN children,and the latest research status of TIN in LN children,aiming to improve clinical understanding of cSLE-related TIN and further strengthen the management of children with LN.
作者
娄丽丽
刘瀚旻
Lou Lii;Liu Hanmin(Department of Pediatrics Key Laboratory of Birth Defects and Reated Diseases of Women and Children(Sichuan University),Ministry of Education,Vascular Remodeling and Developmental Defects Research Unit of West China Institute of Women and Children's Health,West China Second University Hospital,Sichuan University,Chengdu 61004l,Sichuan Province,China)
出处
《中华妇幼临床医学杂志(电子版)》
CAS
2023年第4期373-378,共6页
Chinese Journal of Obstetrics & Gynecology and Pediatrics(Electronic Edition)
基金
国家重点研发计划重点专项(2017YFC0211705)。