First-Line Treatment of NMOSD in AQP4-IgG-Positive Patients
In neuromyelitis optica spectrum disorder (NMOSD), confirmation of Aquaporin-4 immunoglobulin G antibody (AQP4-IgG) positivity in serum defines a high-risk subgroup that requires a specific and prompt treatment strategy, beginning after the very first attack.
Clinical scenario
This protocol is indicated for patients with NMOSD who are confirmed AQP4-IgG positive in serum. This serological finding is clinically significant: long-term immunotherapy must be offered already after the first attack in this population.
Treatment approach — partial overview
The recommended first-choice strategy involves initiating long-term immunotherapy with a monoclonal antibody as monotherapy following the first attack. The structured protocol specifies which agents are recommended when available and accessible, and under what circumstances a different combination approach may apply.
Full agent selection, sequencing criteria, and clinical algorithm available in the complete protocol.
References
DOI: 10.1007/s00415-024-12288-2
- Long-term immunotherapy must be offered to patients with AQP4-IgG-positive NMOSD already after the first attack.
- Long-term immunotherapy in AQP4-IgG-positive NMOSD should be initiated with one of the monoclonal antibodies eculizumab/ravulizumab, inebilizumab, rituximab, or satralizumab, whenever those are available and accessible.
- Long-term immunotherapy with monoclonal antibodies should be started as a monotherapy unless comorbidity warrants a combination with classical immunosuppressive therapies.
- Eculizumab/ravulizumab, inebilizumab, rituximab, satralizumab and tocilizumab are highly effective therapies for AQP4-IgG-positive NMOSD.
View source ↗