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).
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.
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.
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.
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.