Division of Pediatric Rheumatology, Nationwide Children's Hospital, Columbus, OH, 46205, USA.
Division of Nephrology, Nationwide Children's Hospital, Columbus, OH, USA.
Pediatr Rheumatol Online J. 2024 Aug 8;22(1):72. doi: 10.1186/s12969-024-00990-4.
Systemic lupus erythematosus (SLE) is a chronic autoimmune condition that can affect multiple organ systems and is heterogenous in its presentation and response to therapy. When diagnosed in childhood, SLE is associated with increased morbidity and mortality compared to adult SLE, often requiring substantial immunosuppression with the risk of significant side effects. There remains a significant unmet need for new therapies that can improve disease control and reduce glucocorticoid and other toxic medication exposure for patients with severe or refractory disease. The pathogenesis of SLE involves B cell dysregulation and autoantibody production, which are a hallmark of the disease. Currently approved B cell directed therapies often result in incomplete B cell depletion and may not target long-lived plasma cells responsible for SLE autoantibodies. It is hypothesized that by persistently eliminating both B cells and plasmablasts, CAR T therapy can halt autoimmunity and prevent organ damage in patient's refractory to current B cell-depleting treatments. Herein we summarize the current preclinical and clinical data utilizing CAR T cells for SLE and discuss the future of this treatment modality for lupus.
系统性红斑狼疮 (SLE) 是一种慢性自身免疫性疾病,可影响多个器官系统,其表现和对治疗的反应存在异质性。与成人 SLE 相比,儿童时期诊断出的 SLE 发病率和死亡率更高,通常需要大量免疫抑制治疗,且存在严重的副作用风险。对于那些患有严重或难治性疾病的患者,仍然存在着对新疗法的巨大需求,这些新疗法可以改善疾病控制并减少糖皮质激素和其他有毒药物的暴露。SLE 的发病机制涉及 B 细胞失调和自身抗体的产生,这是该疾病的一个标志。目前批准的 B 细胞靶向治疗方法通常导致不完全的 B 细胞耗竭,并且可能无法针对负责 SLE 自身抗体的长寿浆细胞。据推测,通过持续消除 B 细胞和浆母细胞,CAR T 疗法可以阻止自身免疫并防止对当前 B 细胞耗竭治疗有抗药性的患者发生器官损伤。本文总结了目前利用 CAR T 细胞治疗 SLE 的临床前和临床数据,并讨论了这种治疗方式治疗狼疮的未来。