Rituximab Dosing and Immunoglobulin Replacement Therapy in Neuromyelitis Optica Spectrum Disorder: Results from an International Modified Delphi Panel Study
Antonia Lefter1, Tania Kumpfel2, Jacqueline Palace3, Zsolt Illes4, Friedemann Paul5, Georgina Arrambide6, Orhan Aktas7, Ayse Altintas8, Thais Armangue9, Gregor Brecl Jakob10, Andrew Chan11, Kumaran Deiva12, Jelena Drulovic13, Katrin Gross-Paju14, Mario Habek15, Maria Hastermann16, Ellen Iacobaeus17, Maciej Jurynczyk18, Ming Lim19, Sara Mariotto20, Cecilia Rajda21, Ernestina Santos22, Aksel Siva23, Romain Marignier24, Paulus Rommer25, Barbara Willekens26
1Translational Sciences Research Group, Faculty of Medicine and Health Sciences, University of Antwerp, 2Ludwig Maximilian University of Munich, 3John Radcliff Hospital Oxford Univeristy Hospitals Trust, 4Odense University Hospital, University of Southern Denmark, 5Charite Universitatsmedizin in Berlin, 6Cemcat, Vall d´Hebron University Hospital, 7Neurologische Klinik, 8Koc University School of Medicine, 9IDIBAPS-HClinic, 10University Medical Center Ljubljana, University of Ljubljana, 11Bern University Hospital, University of Bern, 12Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris, Paris-Saclay University Hospitals, 13University Clinical Center of Serbia, 14Laane-Tallinna Keskhaigla, 15University Hospital Center Zagreb, School of Medicine, University of Zagreb, 16Max Delbrueck Center for Molecular Medicine and Charité-Universitätmedizin, 17Karolinska Institute, Karolinska University Hospital, 18Institute of Psychiatry and Neurology, 19Evelina London Children`s Hospital at Guy`s and St Thomas` National Health Service Foundation Trust, 20Neurology Unit, University of Verona, 21Department of Neurology, Faculty of Medicine, University of Szeged, 22Santo Antonio University Hospital Centre, University of Porto, 23Istanbul University Cerrahpasa School of Medicine, 24Lyon University Hospital, 25AKH Wien / Univ. Klinik Fuer Neurologie, 26Antwerp University Hospital
Objective:

Identification of real-world clinical practice strategies with rituximab regimes and immunoglobulin replacement therapy (IgRT) in neuromyelitis optica spectrum disorder (NMOSD) among European experts in rare neuroinflammatory diseases (NID).

Background:

Infections are frequent adverse events associated with B cell depleting therapies in NMOSD. Clinical practice involving rituximab de-escalation strategies and IgRT is diverse worldwide.

Design/Methods:

A modified Delphi panel study was conducted from June 2024 until September 2025. Panel members were experts in NID who attended the 2nd and 3rd NMOSD/MOGAD Case-based European Forums held in Tallinn and Prague. The initial voting round featured 8 questions on individual practice with rituximab and 8 questions about IgRT use in AQP4-IgG-positive and double-seronegative NMOSD.

Results:

Seventeen experts in rare NID participated in round 1. Dosing regimens for rituximab vary across countries. Over a third of panelists do not implement either rituximab dose reduction (DR) or extended interval dosing (EID) in NMOSD until infections occur. The most used rituximab DR regime to prevent infections is 500 mg once 6 monthly (47%), considering a standard maintenance regime of 1000 mg once 6 monthly. EID is largely based on CD19+ B cell counts (~65%). Patient`s age, comorbidities, baseline serum immunoglobulin G (IgG) level and prior immunosuppression were considered main risk factors for hypogammaglobulinemia and/or infections driving the decision to perform rituximab DR and/or EID. Immunoglobulin levels are monitored by 76% before start of therapy and 6 monthly thereafter. IgRT to treat secondary hypogammaglobulinemia is based on serum IgG levels (53%) and scheduled 4 weekly (35%) with 0.4 g/kg (65%). The target serum IgG level is 5 g/L for >50% panelists, but varies.

Conclusions:

There are differences in rituximab dosing regimens and IgRT regarding infectious risk management strategies in NMOSD. Notably, there is a lack of data on such strategies, and more research is warranted to inform evidence-based clinical practice recommendations.

Generative AI Usage
No, did not use generative AI in the drafting or editing in this abstract.
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