Department of Rheumatology, Xiangya Hospital, Central South University;Department of Rheumatology, Beijing Hospital, National Center of Gerontology, Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing, 100730, PR China.
Department of Rheumatology, Beijing Hospital, National Center of Gerontology, Institute of Geriatric Medicine, Clincal Immunology Center, Chinese Academy of Medical Sciences, Beijing, 100730, PR China.
J Autoimmun. 2022 Oct;132:102887. doi: 10.1016/j.jaut.2022.102887. Epub 2022 Aug 25.
Immune thrombocytopenia (ITP) is a common hematological manifestation of systemic lupus erythematosus (SLE). The heterogeneity of its clinical characteristics and therapeutic responses reflects a complex pathogenesis. A better understanding of its pathophysiological mechanisms and employing an optimal treatment regimen is therefore important to improve the response rate and prognosis, and avoid unwanted outcomes. Besides glucocorticoids, traditional immunosuppressants (i.e. cyclosporine, mycophenolate mofetil) and intravenous immunoglobulins, new therapies are emerging and promising for the treatment of intractable SLE-ITP, such as thrombopoietin receptor agonists (TPO-RAs), platelet desialylation inhibitors(i.e. oseltamivir), B-cell targeting therapy(i.e. rituximab, belimumab), neonatal Fc receptor(FcRn) inhibitor, spleen tyrosine kinase(Syk) inhibitor and Bruton tyrosine kinase(BTK) inhibitor et al., although more rigorous randomized controlled trials are needed to substantiate their efficacy. In this review, we update our current knowledge on the pathogenesis and treatment of SLE-ITP.
免疫性血小板减少症（ITP）是系统性红斑狼疮（SLE）的一种常见血液学表现。其临床表现和治疗反应的异质性反映了其复杂的发病机制。因此，更好地了解其病理生理机制并采用最佳治疗方案对于提高反应率和预后、避免不良结局非常重要。除了糖皮质激素、传统免疫抑制剂（如环孢素、霉酚酸酯）和静脉注射免疫球蛋白外，新的治疗方法也在不断涌现，并有望治疗难治性 SLE-ITP，如血小板生成素受体激动剂（TPO-RAs）、血小板去唾液酸化抑制剂（如奥司他韦）、B 细胞靶向治疗（如利妥昔单抗、贝利尤单抗）、新生儿 Fc 受体（FcRn）抑制剂、脾酪氨酸激酶（Syk）抑制剂和布鲁顿酪氨酸激酶（BTK）抑制剂等，尽管还需要更严格的随机对照试验来证实它们的疗效。在这篇综述中，我们更新了我们目前对 SLE-ITP 的发病机制和治疗的认识。
