@article{01J717RMKZ2EYN5W9S93Y4CQZX,
  abstract     = {{Background Neuromyelitis 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.

Methods This 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.

Results A 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.

Conclusion Although 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       = {{Siriratnam, Pakeeran and Sanfilippo, Paul and van der Walt, Anneke and Sharmin, Sifat and Foong, Yi Chao and Yeh, Wei Zhen and Zhu, Chao and Khoury, Samia Joseph and Csepany, Tunde and Willekens, Barbara and Etemadifar, Masoud and Ozakbas, Serkan and Nytrova, Petra and Altintas, Ayse and Al-Asmi, Abdullah and Yamout, Bassem and Laureys, Guy and Patti, Francesco and Simo, Magdolna and Surcinelli, Andrea and Foschi, Matteo and McCombe, Pamela A and Alroughani, Raed and Sánchez-Menoyo, José Luis and Turkoglu, Recai and Soysal, Aysun and Lechner Scott, Jeanette and Kalincik, Tomas and Butzkueven, Helmut and Jokubaitis, Vilija and Huda, Saif and Monif, Mastura and Van Hijfte, Liesbeth and MSBASE study group, [missing]}},
  issn         = {{0022-3050}},
  journal      = {{JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY}},
  keywords     = {{MULTIPLE SCLEROSIS,NEUROIMMUNOLOGY,IMMUNOLOGY,MEDICINE,HEALTH ECONOMICS,OPTICA SPECTRUM DISORDER,NEUROMYELITIS-OPTICA,DOUBLE-BLIND,EFFICACY,SAFETY,AZATHIOPRINE,SATRALIZUMAB,RITUXIMAB,OUTCOMES}},
  language     = {{eng}},
  number       = {{4}},
  pages        = {{361--369}},
  title        = {{Predictors of relapse risk and treatment response in AQP4-IgG positive and seronegative NMOSD : a multicentre study}},
  url          = {{http://doi.org/10.1136/jnnp-2024-334090}},
  volume       = {{96}},
  year         = {{2025}},
}

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