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International Delphi Consensus on the Management of AQP4-IgG+ NMOSD: Recommendations for Eculizumab, Inebilizumab,
Friedemann Paul1, Romain Marignier2, Jacqueline Palace2
1From the Experimental and Clinical Research Center (F.P.), Max Delbrueck Center for Molecular Medicine and Charité Universitaetsmedizin Berlin, Germany; Hospices Civils de Lyon (R.M.), Service de Neurologie, Sclérose en Plaques, Pathologies de la Myéline et Neuro Inflammation, and Centre de Référence des Maladies Inflammatoires Rares du Cerveau et de la Moelle (MIRCEM), Hôpital Neurologique Pierre Wertheimer, Bron; Centre des Neurosciences de Lyon-FORGETTING Team (R.M.), INSERM 1028 et CNRS UMR5292; Université Claude Bernard Lyon 1 (R.M.), France; John Radcliff Hospital (J.P.); Clinical Neurology Oxford University (J.P.), Oxford, United Kingdom; Servei de Neurologia-Neuroimmunologia (G.A.), Centre d'Esclerosi Múltiple de Catalunya (Cemcat); Vall d'Hebron Institut de Recerca (G.A.), Vall d'Hebron Hospital Universitari; Universitat Autònoma de Barcelona (G.A.), Spain; Departments of Regional Health Research and Molecular Medicine (N.A.), University of Southern Denmark, Odense, Denmark; Department of Neurology (N.A.), Slagelse Hospital, Denmark; Programs in Neuroscience and Immunology (J.L.B.), Departments of Neurology and Ophthalmology, University of Colorado Anschutz Medical Campus, Aurora; Department of Neurology (B.A.C.C.), UCSF Weill Institute for Neurosciences, University of California San Francisco; Department of Neurology (J.D.S.), Hôpitaux Universitaires de Strasbourg; INSERM U1119 Biopathologie de la Myéline (J.D.S.), Neuroprotection et Stratégies Thérapeutique; Clinical Investigation Center (J.D.S.), Hôpitaux Universitaires de Strasbourg, France; Department of Multiple Sclerosis Therapeutics (K.F.), Fukushima Medical University School of Medicine, and Multiple Sclerosis and Neuromyelitis Optica Center, Southern TOHOKU Research Institute for Neuroscience, Koriyama, Japan; Department of Neurology (H.J.K.), Research Institute and Hospital of National Cancer Center, Goyang, South Korea; Oxford PharmaGenesis Ltd (R.H., L.L.); Department of Neurology (S.H.), Walton Centre NHS Foundation Trust, Liverpool, United Kingdom; Medical Research Center (N.K.), Marrakesh Medical School, Cadi Ayyad University; Neurology Department (N.K.), University Teaching Hospital Mohammed VI, Marrakesh, Morocco; Department of Neurology (I.K.), St Josef-Hospital, Ruhr-University Bochum; Marianne-Strauß-Klinik (I.K.), Behandlungszentrum Kempfenhausen für Multiple Sklerose Kranke, Berg, Germany; Department of Neurology (S.K.), Graduate School of Medicine, Chiba University, Japan; CIEM MS Research Center (M.L.-P.), Federal University of Minas Gerais, Belo Horizonte, Brazil; John Radcliffe Hospital (M.I.L.), University of Oxford, United Kingdom; KS Hegde Medical Academy Director (L.P.), Center for Advanced Neurological Research, Nitte University, Mangalore, India; Neurology (S.J.P.), Laboratory Medicine and Pathology, Center for Multiple Sclerosis and Autoimmune Neurology, Mayo Clinic, Rochester, MN; Department of Neurology and Rare Disease Center (C.Q.), National Center for Neurological Disorders, Huashan Hospital, Shanghai Medical College, Fudan University, China; Translational Neuroimmunology Group (S.R.), Kids Neuroscience Centre, and Brain and Mind Centre, Sydney Medical School, Faculty of Medicine and Health, University of Sydney; Department of Neurology (S.R.), Concord Hospital, Australia; Division of Neurology (D.R.), Department of Medicine, University of Toronto, Ontario, Canada; Neuroimmunology and Multiple Sclerosis Unit (A.S.), Service of Neurology, Hospital Clinic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), and Universitat de Barcelona, Spain; School of Medicine (D.K.S.), Pontifical Catholic University of Rio Grande do Sul, Porto Alegre, Brazil; and Department of Neurology and Laboratory of Neuroimmunology and The Agnes-Ginges Center for Neurogenetics (A.V.-D.), Hadassah-Medical Center, Ein-Kerem, Faculty of Medicine, Hebrew University of Jerusalem, Israel. friedemann.paul@charite.de.
Background And Objectives:
Neuromyelitis optica spectrum disorder (NMOSD) is a rare debilitating autoimmune disease of the CNS. Three monoclonal antibodies were recently approved as maintenance therapies for aquaporin-4 immunoglobulin G (AQP4-IgG)-seropositive NMOSD (eculizumab, inebilizumab, and satralizumab), prompting the need to consider best practice therapeutic decision-making for this indication. Our objective was to develop validated statements for the management of AQP4-IgG-seropositive NMOSD, through an evidence-based Delphi consensus process, with a focus on recommendations for eculizumab, inebilizumab, and satralizumab.
Methods:
We recruited an international panel of clinical experts in NMOSD and asked them to complete a questionnaire on NMOSD management. Panel members received a summary of evidence identified through a targeted literature review and provided free-text responses to the questionnaire based on both the data provided and their clinical experience. Responses were used to generate draft statements on NMOSD-related themes. Statements were voted on over a maximum of 3 rounds; participation in at least 1 of the first 2 rounds was mandatory. Panel members anonymously provided their level of agreement (6-point Likert scale) on each statement. Statements that failed to reach a predefined consensus threshold (≥67%) were revised based on feedback and then voted on in the next round. Final statements were those that met the consensus threshold (≥67%).
Results:
The Delphi panel comprised 24 experts, who completed the Delphi process in November 2021 after 2 voting rounds. In round 1, 23/25 statements reached consensus and were accepted as final. The 2 statements that failed to reach consensus were revised. In round 2, both revised statements reached consensus. Twenty-five statements were agreed in total: 11 on initiation of or switching between eculizumab, inebilizumab, and satralizumab; 3 on monotherapy/combination therapy; 7 on safety and patient population considerations; 3 on biomarkers/patient-reported outcomes; and 1 on research gaps.
Discussion:
An established consensus method was used to develop statements relevant to the management of AQP4-IgG-seropositive NMOSD. These international statements will be valuable for informing individualized therapeutic decision-making and could form the basis for standardized practice guidelines.
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