摘要
Neuromyelitis optica(NMO)/NMO spectrum disorder(NMOSD)is a chronic,recurrent,antibodymediated,inflammatory demyelinating disease of the central nervous system,characterized by optic neuritis and transverse myelitis.The binding of NMO-IgG with astrocytic aquaporin-4(AQP4)functions directly in the pathogenesis of>60%of NMOSD patients,and causes astrocyte loss,secondary inflammatory infiltration,demyelination,and neuron death,potentially leading to paralysis and blindness.Current treatment options,including immunosuppressive agents,plasma exchange,and B-cell depletion,are based on small retrospective case series and open-label studies.It is noteworthy that monoclonal antibody(mAb)therapy is a better option for autoimmune diseases due to its high efficacy and tolerability.Although the pathophysiological mechanisms of NMOSD remain unknown,increasingly,therapeutic studies have focused on mAbs,which target B cell depletion,complement and inflammation cascade inactivation,bloodbrain-barrier protection,and blockade of NMO-IgG-AQP4 binding.Here,we review the targets,characteristics,mechanisms of action,development,and potential efficacy of mAb trials in NMOSD,including preclinical and experimental investigations.
基金
This review was supported by the National Natural Science Foundation of China(81571596 and 81601044)
the National Key R&D Program of China(2017YFC1701300)
and Fundamental Research Funds for the Central Universities,China(GK201701009).
