Transitioning to a C5 inhibitor for aquaporin-4 antibody–positive neuromyelitis optica spectrum disorder intolerant of anti-CD19 therapy: a case report

Abstract

Introduction

Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory disease of the central nervous system associated with pathogenic aquaporin-4 (AQP4) immunoglobulin G (IgG) antibodies. Without treatment, patients experience recurrent attacks, which can lead to significant disability from accumulated neurological damage. Agents targeting CD19, interleukin-6 receptor, and complement C5 have been approved for treatment of patients with AQP4-IgG+ NMOSD. Real-world data on patients transitioning from an anti-CD19 therapy to a C5 inhibitor are limited, and there are no published data on transitioning between C5 inhibitors, eculizumab and ravulizumab.

Methods

We describe a patient with AQP4-IgG+ NMOSD who transitioned from inebilizumab to eculizumab after developing hypogammaglobulinemia and infections; the patient later transitioned to ravulizumab for less frequent dosing.

Results

Throughout treatment with inebilizumab, eculizumab, and ravulizumab, the patient remained clinically and radiographically stable.

Conclusion

This complex case highlights the importance of monitoring and managing treatment-related complications associated with anti-CD19 therapy and key factors to consider when transitioning from B-cell–depleting therapy to complement inhibition therapy.