• Pakeeran Siriratnam, Paul Sanfilippo, Anneke van der Walt, Sifat Sharmin, Yi Chao Foong, Wei Zhen Yeh, Chao Zhu, Samia Joseph Khoury, Tunde Csepany, Barbara Willekens, Masoud Etemadifar, Serkan Ozakbas, Petra Nytrova, Ayse Altintas, Abdullah Al-Asmi, Bassem Yamout, Guy Laureys, Francesco Patti, Magdolna Simo, Andrea Surcinelli, Matteo Foschi, Pamela A McCombe, Raed Alroughani, José Luis Sánchez-Menoyo, Recai Turkoglu, Aysun Soysal, Jeanette Lechner Scott, Tomas Kalincik, Helmut Butzkueven, Vilija Jokubaitis, Saif Huda, Mastura Monif, and MSBASE study group.
• Department of Neuroscience, School of Translational Medicine, Monash University, Melbourne, Victoria, Australia.
• J. Neurol. Neurosurg. Psychiatr. 2024 Sep 4.

BackgroundNeuromyelitis optica spectrum disorder (NMOSD) can be categorised into aquaporin-4 antibody (AQP4-IgG) NMOSD or seronegative NMOSD. While our knowledge of AQP4-IgG NMOSD has evolved significantly in the past decade, seronegative NMOSD remains less understood. This study aimed to evaluate the predictors of relapses and treatment responses in AQP4-IgG NMOSD and seronegative NMOSD.MethodsThis was a multicentre, international, retrospective cohort study using the MSBase registry. Recurrent relapse risk was assessed using an Andersen-Gill model and risk of first relapse was evaluated using a Cox proportional hazards model. Covariates that putatively influence relapse risk included demographic factors, clinical characteristics and immunosuppressive therapies; the latter was assessed as a time-varying covariate.ResultsA total of 398 patients (246 AQP4-IgG NMOSD and 152 seronegative NMOSD) were included. The AQP4-IgG NMOSD and seronegative NMOSD patients did not significantly differ by age at disease onset, ethnicity or annualised relapse rate. Both low-efficacy and high-efficacy immunosuppressive therapies were associated with significant reductions in recurrent relapse risk, with notably greater protection conferred by high-efficacy therapies in both AQP4-IgG NMOSD (HR 0.27, 95% CI 0.15 to 0.49, p<0.001) and seronegative NMOSD (HR 0.21, 95% CI 0.08 to 0.51, p<0.001). Longer disease duration (HR 0.97, 95% CI 0.95 to 0.99, p<0.001) and male sex (HR 0.52, 95% CI 0.34 to 0.84, p=0.007) were additional protective variables in reducing the recurrent relapse risk for the AQP4-IgG NMOSD group.ConclusionAlthough further studies are needed to improve our understanding of seronegative NMOSD, our findings underscore the importance of aggressive treatment with high-efficacy immunotherapies in both NMOSD subtypes, regardless of serostatus.© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.

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