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

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

This protocol covers management of neuromyelitis optica spectrum disorder (NMOSD) in patients who are positive for aquaporin-4 immunoglobulin G antibody (AQP4-IgG) in serum — a clinically distinct subgroup for whom long-term immunotherapy is indicated from the outset.

Clinical Scenario

Aquaporin-4 immunoglobulin G antibody (AQP4-IgG) is detectable in serum. Long-term immunotherapy must be offered to patients with AQP4-IgG-positive NMOSD already after the first attack.

Treatment Approach — Partial Overview

The protocol centres on add-on combination therapy incorporating a monoclonal antibody together with select immunosuppressive agents, with further options outlined for cases that are refractory or where first-choice agents cannot be used.

Full regimen, agent selection, sequencing, and all dosing details are 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.

However, immunosuppressive therapy may be offered as add-on therapy to patients refractory after treatment with > 2 monoclonal antibodies in succession—or in countries where alternative monoclonal antibodies are not available.

In children or in case of contraindications to other therapies intravenous immunoglobulins may be used; methotrexate and tacrolimus may be used if other therapies are not available.

Finally, intermittent PE or IA combined with or without concomitant immunosuppressive therapy has also been shown to prevent attacks in severe NMOSD in individual case reports.

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