Marchi-Silva Rodrigo, De Aquino Bruna Martins, Londe Ana Carolina, Mazzola Taίs Nitsch, Julio Paulo Rogério, Wampler Muskardin Theresa, Appenzeller Simone
Medical Pathophysiology Graduate Program, School of Medical Science, Universidade Estadual de Campinas, Campinas, Brazil.
Autoimmunity Lab, School of Medical Science, Universidade Estadual de Campinas, Campinas, Brazil.
Int J Nephrol Renovasc Dis. 2025 Jan 13;18:1-12. doi: 10.2147/IJNRD.S405789. eCollection 2025.
Approximately one in five patients with systemic lupus erythematosus (SLE) has disease-onset during childhood (cSLE). Lupus nephritis is more common in cSLE than adult-onset SLE and is associated with significant and increased morbidity and mortality. In this article, we review lupus nephritis in cSLE, including pathogenesis, diagnosis, biomarkers, and management through PUBMED search between July and December 2024. Diagnosis of lupus nephritis is made in 93% of cSLE patients during the first 2 years of disease. The majority of patients have active disease in other organs, and nephrotic range proteinuria and hypertension is frequently observed at diagnosis. Class III and IV are observed in over 50% of renal biopsies and progression to end-stage renal disease varies across cohorts. Major progress made in recent years includes adjustment of the proportion of fibrous crescents when scoring nephritis in cSLE to better discriminate kidney disease outcomes, and development of non-invasive biomarkers to identify renal disease activity and damage. It is anticipated that accurate non-invasive biomarkers will foster multicenter studies and help identify new treatment approaches to improve outcomes in cSLE nephritis.
大约五分之一的系统性红斑狼疮（SLE）患者在儿童期发病（儿童期起病的SLE，cSLE）。狼疮性肾炎在cSLE中比成人起病的SLE更常见，并且与显著增加的发病率和死亡率相关。在本文中，我们通过在2024年7月至12月期间进行的PubMed搜索，对cSLE中的狼疮性肾炎进行综述，内容包括发病机制、诊断、生物标志物和治疗。93%的cSLE患者在疾病的前两年内被诊断为狼疮性肾炎。大多数患者在其他器官也有活动性疾病，并且在诊断时经常观察到肾病范围的蛋白尿和高血压。超过50%的肾活检显示为III级和IV级，不同队列中进展至终末期肾病的情况各不相同。近年来取得的主要进展包括在对cSLE中的肾炎进行评分时调整纤维性新月体的比例，以更好地区分肾脏疾病的预后，以及开发非侵入性生物标志物以识别肾脏疾病的活动和损伤。预计准确的非侵入性生物标志物将促进多中心研究，并有助于确定新的治疗方法以改善cSLE肾炎的预后。
