An international panel of clinical experts reached a consensus on statements regarding the management of patients with neuromyelitis optica spectrum disorder (NMOSD) who are seropositive for aquaporin-4 immunoglobulin G (AQP4-IgG+). The statements have been published in Neurology: Neuroimmunology & Neuroinflammation.
Twenty-five consensus statements were agreed upon using the Delphi process, including 11 statements related to the initiation of or switching between eculizumab, inebilizumab, and satralizumab, 3 on monotherapy/combination therapy, 7 pertaining to safety and patient population considerations, 3 on biomarkers and patient outcomes, and 1 on research gaps.
“In conclusion, the consensus statements developed in this Delphi process seek to address an unmet need for providing recommendations on the use of eculizumab, inebilizumab, and satralizumab to treat patients with AQP4-IgG–seropositive NMOSD,” the authors wrote.
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The statements agreed that eculizumab, inebilizumab, or satralizumab may be initiated at diagnosis, after the first attack, or after relapse due to failure of existing treatments in adult patients. Satralizumab may be initiated in adolescent patients (12 years or older) as well. Eculizumab or inebilizumab may be considered in adolescents only in the case of severe disease that is refractory to satralizumab.
The choice between the 3 treatments should be informed by the patient’s preferences related to dosing frequency, the route of administration, and acceptance of potential safety risks during treatment. The presence of other comorbidities should also be considered.
In addition, the patient’s response to previous maintenance therapy should be examined. If previous treatments failed, a new treatment with a different mode of action should be considered. Patients who are free of relapses or tolerability issues while using off-label immunosuppressants do not need to initiate treatment with eculizumab, inebilizumab, or satralizumab.
Monotherapy is preferred, but combination therapy with eculizumab or satralizumab combined with immunosuppressant therapy may be considered in patients already receiving immunosuppressants. If combination therapy is utilized, patients should be closely monitored for side effects and the immunosuppressant therapy should be slowly tapered.
Switching of therapies should take into consideration the occurrence of serious treatment-related adverse events, a severe relapse while on treatment, and the patient’s preferences. When switching, the new therapy may be initiated immediately, but the mechanism and duration of action of the previous treatment should be considered.
Patients receiving treatment should be monitored for infections, and some specific patient populations should be monitored more than twice a year. To aid in lowering the risk of infections, patients should be current on all vaccinations before initiating new biologic therapies.
The panel acknowledged that while serum glial fibrillary acidic protein (GFAP) and serum neurofilament light chain (NfL) have been shown to be biomarkers of NMOSD activity, more research is needed to support their use in treatment decision-making. In addition, more research is required to support the use of health-related quality of life outcomes for treatment decision-making.
The panel agreed that further research is needed in several areas including biomarkers, imaging, head to head evidence, and long-term outcomes of treatment.
Reference
Paul F, Marignier R, Palace J, et al. International Delphi consensus on the management of AQP4-IgG+ NMOSD: recommendations for eculizumab, inebilizumab, and satralizumab. Neurol Neuroimmunol Neuroinflamm. Published online May 31, 2023. doi:10.1212/NXI.0000000000200124
