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Neuromyelitis optica spectrum disorders (NMOSDs) have the characteristics of high relapse rate and high disability rate. The neurological deficits would become more and more serious with increasing relapse. Using corticosteroids pulse therapy, intravenous immunoglobulin or plasma exchange to reduce inflammation and inhibit neurological dysfunction is the main treatment principle of NMOSDs in the acute phase; drugs to prevent relapse of NMOSDs are mainly immunosuppressants, such as azathioprine, and targeted agents, such as CD19 monoclonal antibody, C5 complement, interleukin⁃6⁃receptor targeted antibodies and a proliferation⁃inducing ligand (APRIL)/transmembrance activator and calcium⁃modulator and cyclophilin ligand interactor (TACI) targeted therapy. Some targeted agents have been approved for clinical use. This article intended to review the targeted agents for NMOSDs patients for the purpose of improving the understanding of therapeutic strategies of NMOSDs. DOI: 10.3969/j.issn.1672-6731.2020.10.001