Neuromyelitis optica spectrum disorder
ICD-10 G36.0 · ICD-11 8A43

Treatment of Neuromyelitis Optica Spectrum Disorder in AQP4-IgG Positive Patients

Neuromyelitis optica spectrum disorder (NMOSD) in patients with aquaporin-4 immunoglobulin G antibody (AQP4-IgG) positivity in serum represents a serologically defined subgroup that carries distinct therapeutic implications from the first attack onward.

Clinical scenario

AQP4-IgG positivity in serum identifies patients for whom long-term immunotherapy must be offered already after the first attack. This protocol defines the structured approach for this specific population.

Treatment approach (partial overview)

The protocol specifies a biologic, monoclonal antibody-based therapeutic strategy as the primary approach. Criteria for agent selection and the role of alternative options when biologicals are unavailable are addressed in the full regimen.

Full drug selection, sequencing, and criteria available in the complete protocol →
Instant Access to Structured Evidence-Based Regimens

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.

In case of treatment failure with a monoclonal antibody, therapy should be switched to another monoclonal antibody, preferably with a different mode of action.

Conventional immunosuppressive therapies (azathioprine, mycophenolate mofetil, oral glucocorticoids) may be used but are considered less effective than biologicals.

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