Immunosuppressants For Neuromyelitis Optica：a Descriptive Systematic Review

Objective：To assess the effectiveness and safety of immunosuppressants inpeople with neuromyelitis optica.Methods: A systematic literature search of the CENTRAL, MEDLINE,EMBASE, MS Group Register and other network resources was performed.Original studies on clinical efficacy of immunosuppressants were included. Themethodology quality of the relevant published data was evaluated and adescriptive analysis was performed.Results: Twenty-two observational studies or reports met the inclusioncriteria, including290patients. The immunosuppressants included areazathioprine, rituximab, mycophenolate mofetil, mitoxantrone,cyclophosphamide and methotrexate. These studies were quite heterogeneous andgenerally of low validity. Nevertheless, according to currently available data,disability improved or stabilized in68.8%patients who were treated withazathioprine. And the percentages of patients who were treated with rituximab,mycophenolate mofetil, and mitoxantrone are76.7%,92.0%, and89.3%. Themedian annualized relapse rate got reduced in90.6%patients who were treatedwith azathioprine. And the percentages of patients who were treated withrituximab, mycophenolate mofetil, and mitoxantrone are84.0%,80.0%, and92.9%. The cases treated with cyclophosphamide and methotrexate are not enough for statistical analysis. The immunosuppressants are well tolerated,except the lymphoma after azathioprine in3patients and the posterior reversibleencephalopathy syndrome (PRES) after rituximab infusion in a woman.Conclusions: In neuromyelitis optica, treatment with immunosuppressantsappears to reduce the frequency of attacks, with subsequent stabilization orimprovement in disability. As first-line therapy, azathioprine is recommended. Iffirst-line treatment is ineffective, alternative immunosuppressive therapies, suchas rituximab, mycophenolate mofetil and mitoxantrone need to be considered.There is lack of high-quality evidence to estimate the relative treatment effects ofthe immunosuppressants in neuromyelitis optica. Therefore, prospectiverandomized controlled comparative trials are necessary.