补体靶向药物治疗AQP4-IgG阳性视神经脊髓炎谱系疾病的进展

Progress of Complement-targeting Drugs in AQP4-IgG-positive Neuromyelitis Optica Spectrum Disorders

视神经脊髓炎谱系疾病(NMOSD)是一种严重的炎症性脱髓鞘疾病,75%NMOSD患者体内存在针对中枢神经系统水通道蛋白4(AQP4)的致病性抗体(AQP4-IgG)。补体系统是一组存在于人和动物血清及组织液、细胞膜表面的经活化后具有生物活性的蛋白质。近年来,随着对NMOSD致病机制的研究不断深入,补体系统被发现在AQP4-IgG阳性NMOSD发生发展中具有重要作用,并且参与机体免疫调节。目前,针对自身免疫性疾病的补体靶向药物陆续被研发,部分已应用于AQP4-IgG阳性NMOSD患者的治疗。文中就AQP4-IgG阳性NMOSD补体靶向药物治疗的研究进展展开综述。

Neuromyelitis optica spectrum disorders(NMOSD)is a serious inflammatory demyelinating disease.Pathogenic antibody against aquaporin protein-4(AQP4)in central nervous system was presented in 75%of patients.Complement system is a group of proteins which are biologically activated existing in human and animal serum,tissue fluid and cell membrane surface.With the progress about the pathogenic mechanism of NMOSD,complement system has been found to play an important role in the occurrence and development of AQP4-IgG-positive NMOSD,and participate in the immune regulation.Complement-targeting drugs for autoimmune diseases have been successfully developed,and some of them have been applied to the treatment of AQP4-IgG-positive NMOSD.The research progress of complement-targeting drug therapy in AQP4-IgG-positive NMOSD were reviewed.