Ivana Stojkic, Lauren Harper, Samantha Coss, Mahmoud Kallash, Kyla Driest, Margaret Lamb, Stacy P Ardoin, Shoghik Akoghlanian
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引用次数: 0
摘要
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各不相同。与成人系统性红斑狼疮相比,在儿童期确诊的系统性红斑狼疮会增加发病率和死亡率,通常需要大量的免疫抑制,并有可能产生严重的副作用。对于严重或难治性疾病患者来说,新疗法能改善疾病控制并减少糖皮质激素和其他有毒药物的暴露,但这种需求仍未得到满足。系统性红斑狼疮的发病机制涉及 B 细胞失调和自身抗体的产生,这是该病的一个特征。目前已获批准的 B 细胞靶向疗法通常无法完全清除 B 细胞,也可能无法靶向产生系统性红斑狼疮自身抗体的长效浆细胞。据推测,通过持续清除B细胞和浆细胞,CAR T疗法可以阻止自身免疫,并防止目前B细胞清除疗法难治患者的器官损伤。在此,我们总结了目前利用CAR T细胞治疗系统性红斑狼疮的临床前和临床数据,并讨论了这种狼疮治疗方式的未来。
CAR T cell therapy for refractory pediatric systemic lupus erythematosus: a new era of hope?
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.
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.