Immunoadsorption as maintenance therapy for refractory neuromyelitis optica spectrum disorder

Area of Science:

• Neuroimmunology
• Autoimmune disorders of the central nervous system
• Therapeutic apheresis

Background:

• Neuromyelitis Optica Spectrum Disorder (NMOSD) is a rare autoimmune CNS disease targeting optic nerves and spinal cord, often associated with anti-aquaporin-4 (AQP4) IgG autoantibodies.
• Immunoadsorption (IA) is effective for NMOSD attacks, but long-term data for preventing relapses and progression are limited.

Observation:

• This case study retrospectively analyzed two female NMOSD patients refractory to conventional immunotherapies.
• Both patients received tryptophan IA (Tr-IA) for acute attacks and subsequently underwent biweekly maintenance Tr-IA.

Findings:

• Patient 1 (AQP4-IgG seropositive) experienced significantly fewer attacks and stable disability (EDSS 5.0) with maintenance Tr-IA over 3.1 years.
• Patient 2 (AQP4-IgG seronegative) remained attack-free for 5.3 years with Tr-IA, showing improved disability (EDSS 5.0) and ambulation.
• Both patients tolerated Tr-IA well, with no serious adverse events reported during long-term treatment.

Implications:

• Maintenance Tr-IA appears to be a safe and effective strategy for achieving clinical stabilization in highly active NMOSD patients unresponsive to standard therapies.
• This approach may offer a viable long-term treatment option for managing refractory NMOSD, reducing relapse rates and disability progression.