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国家卫生健康委员会
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【摘要】系统性红斑狼疮是一种以自身抗体形成和免疫复合物沉积为特征的复杂的自身免疫性疾病。狼疮性肾炎(LN)是系统性红斑狼疮最严重的临床表现之一,累及大多数患者,其发病机制涉及环境因素、激素调节、易感基因(包括控制凋亡率的基因)、免疫复合物清除、细胞信号转导以及影响炎性反应基因之间的复杂相互作用。近年来,由于早期诊断以及糖皮质激素联合环磷酰胺、霉酚酸酯等免疫抑制剂综合治疗的应用,LN患者生存率明显提高,但难治性LN患者仍然面临肾脏反应率低、维持缓解期长、复发率高及不良反应多等诸多问题,新型药物及治疗方案不断被探索。本文对难治性LN的治疗进展进行综述。
【Abstract】Systemic lupus erythematosus(SLE) is a complex autoimmune disease characterized by autoantibody formation and immune complex deposition. Lupus nephritis(LN) is one of the most serious manifestations of SLE affecting most patients. Its pathogenesis involves environmental factors, hormone regulation, susceptible genes(including genes that control apoptosis), clearance of immune complexes, cell signal transduction, and influence on the interaction between inflammatory response genes. In recent years, owing to early diagnosis and combined treatment with glucocorticoids, cyclophosphamide, mycophenolate and other immunosuppressive agents, the survival rate of LN patients have been significantly improved, but patients with refractory LN still facing great challenges, such as low renal response rate, long remission period, high risk of recurrence and adverse reactions. New drugs and treatment schemes have been explored. This article reviewed the research progress of treatment for refractory LN.
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