International Delphi Consensus on the Management of AQP4-IgG+ NMOSD: Recommendations for Eculizumab, Inebilizumab, and Satralizumab

Friedemann Paul¹, Romain Marignier², Jacqueline Palace², Georgina Arrambide², Nasrin Asgari², Jeffrey L Bennett², Bruce Anthony Campbell Cree², Jérôme De Sèze², Kazuo Fujihara², Ho Jin Kim², Rebecca Hornby², Saif Huda², Najib Kissani², Ingo Kleiter², Satoshi Kuwabara², Marco Lana-Peixoto², Lisa Law², M Isabel Leite², Lekha Pandit², Sean J Pittock², Chao Quan², Sudarshini Ramanathan², Dalia Rotstein², Albert Saiz², Douglas Kazutoshi Sato², Adi Vaknin-Dembinsky²

Affiliations

¹ From 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.
² From 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.

PMID: 37258412
PMCID: PMC10231913
DOI: 10.1212/NXI.0000000000200124

International Delphi Consensus on the Management of AQP4-IgG+ NMOSD: Recommendations for Eculizumab, Inebilizumab, and Satralizumab

Friedemann Paul et al. Neurol Neuroimmunol Neuroinflamm. 2023.

. 2023 May 31;10(4):e200124.

doi: 10.1212/NXI.0000000000200124. Print 2023 Jul.

Authors

Affiliations

¹ From 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.
² From 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.

PMID: 37258412
PMCID: PMC10231913
DOI: 10.1212/NXI.0000000000200124

Abstract

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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Conflict of interest statement

F. Paul served on the scientific advisory boards of Novartis and MedImmune; received travel funding and/or speaker honoraria from Bayer, Novartis, Biogen, Teva, Sanofi-Aventis/Genzyme, Merck Serono, Alexion, Chugai, MedImmune, and Shire; is an associate editor of Neurology: Neuroimmunology & Neuroinflammation; is an academic editor of PLoS ONE; consulted for Sanofi Genzyme, Biogen, MedImmune, Shire, and Alexion; received research support from Bayer, Novartis, Biogen, Teva, Sanofi-Aventis/Geynzme, Alexion, and Merck Serono; and received research support from the German Research Council, Werth Stiftung of the City of Cologne, German Ministry of Education and Research, Arthur Arnstein Stiftung Berlin, EU FP7 Framework Program, Arthur Arnstein Foundation Berlin, Guthy-Jackson Charitable Foundation, and NMSS; J. Palace has received support for scientific meetings and honorariums for advisory work From Merck Serono, Novartis, Chugai, Alexion, Roche, Medimmune, Argenx, UCB, Mitsubishi, Amplo, Janssen, Sanofi. Grants from Alexion, Roche, Medimmune, UCB, and Amplo biotechnology; has patent ref P37347WO and licence agreement Numares multimarker MS diagnostics Shares in AstraZenica; and acknowledges partial funding by highly specialised services NHS England; R. Marignier reports personal fees from Viela Bio, Roche, and UCB and nonfinancial support from Viela Bio, Merck, Biogen, and Roche, outside the submitted work. AC-C reports funding from the Instituto de Salud Carlos III (Spain) JR19/00007; S. Ramanathan has received research funding from the National Health and Medical Research Council (Australia), the Petre Foundation, the Brain Foundation (Australia), the Royal Australasian College of Physicians, and the University of Sydney. She is supported by an NHMRC Investigator Grant (GNT2008339). She serves as a consultant on an advisory board for UCB and Limbic Neurology and has been an invited speaker for Biogen, Excemed, and Limbic Neurology; M. Lana-Peixoto reports no disclosures relevant to the manuscript; L. Law is an employee of Oxford PharmaGenesis; D.K. Sato reports grants from Conselho Nacional de Desenvolvimento Científico e Tecnológico, Fundação de Amparo à Pesquisa do Estado do Rio Grande do Sul, TEVA, and Merck and personal fees from TEVA, Merck, Biogen, Roche, and Viela Bio, outside the submitted work. KS reports personal fees from Biogen, Novartis, Merck, Roche, Celgene, and TG Therapeutics and grants from Merck and Roche, outside the submitted work; C. Quan received travel funding and/or speaker honoraria from Sanofi Genzyme, Novatis, Roche, Biogen, and Bristol Myers Squibb; is on the editorial board for Neuroimmunology Reports; and received research support from Novatis; N. Asgari reports no disclosures; J. De Seze has done some consulting and served on the board for Roche; I. Kleiter has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Alexion, Almirall, Biogen, Celgene, Hexal, Horizon, Merck, and Roche/Chugai; L. Pandit has received Speaker honorarium from Biogen and consulted for Biogen, Novartis, and Sanofi. She is listed as an inventor of a live cell-based assay for AQP4-IgG for which her university (Nitte university) holds a patent (No: 202141055841A); A. Vaknin-Dembinsky reported grants from F. Hoffmann-La Roche Ltd; personal fees from Roche, Biogen, Genzyme Sanofi, Merck, and Novartis; and grants from Merck and the Ministry of Health of Israel outside the submitted work. No other disclosures were reported; K. Fujihara serves on scientific advisory boards or as a consultant for Biogen, Mitsubishi-Tanabe, Novartis, Chugai, Roche, Alexion, VielaBio/Horizon Therapeutics, UCB, Merck Biopharma, Japan Tobacco, Argenx, and Abbvie; has received funding for travel or speaker honoraria from Chugai, Roche, Biogen, Novartis, Alexion, Teijin, Mitsubishi-Tanabe, AsahiKasei, Eisai, Takeda, and Bayer; serves on editorial boards of Clinical and Experimental Neuroimmunology, Frontiers in Neurology, Neurology: Neuroimmunology and Neuroinflammation, MS, MS and Related Disorders and Neuroimmunology Reports and advisory board of Sri Lanka journal of Neurology; and has been funded by the Grants-in-Aid for Scientific Research from the Ministry of Education, Science and Technology of Japan and by the Grants-in-Aid for Scientific Research from the Ministry of Health, Welfare and Labor of Japan; S. Kuwabara reports no disclosures relevant to the manuscript; N. Kissani reports no disclosures relevant to the manuscript; H.J. Kim received a grant from the National Research Foundation of Korea and research support from Aprilbio and Eisai; received consultancy/speaker fees from Alexion, Aprilbio, Altos Biologics, Biogen, Celltrion, Daewoong, Eisai, GC Pharma, HanAll BioPharma, Handok, Horizon Therapeutics (formerly Viela Bio), Kolon Life Science, MDimune, Mitsubishi Tanabe Pharma, Merck Serono, Novartis, Roche, Sanofi Genzyme, Teva-Handok, and UCB; is a coeditor for the MS Journal; and an associated editor for the Journal of Clinical Neurology; A. Saiz received compensation for consulting services and speaker honoraria from Merck, Biogen, Sanofi, Novartis, Roche, Janssen, Alexion, and Horizon Therapeutics; R. Hornby is an employee of Oxford PharmaGenesis; G. Arrambide has received speaking honoraria, compensation for consulting services, or participation in advisory boards from Sanofi, Merck, Roche, and Horizon Therapeutics and travel support for scientific meetings from Novartis, Roche, and ECTRIMS; is the editor for Europe of the MS Journal—Experimental, Translational and Clinical, and is a member of the International Women in MS (iWiMS) Network executive committee and of the European Biomarkers in MS (BioMS-eu) Consortium steering committee; S. Huda reports no disclosures relevant to the manuscript; M.I. Leite is funded by the NHS (Myasthenia and Related Disorders Service and National Specialised Commissioning Group for Neuromyelitis Optica, UK) and by the University of Oxford, UK. She has been awarded research grants from the UK association for patients with myasthenia—Myaware and the University of Oxford. She has received speaker honoraria or travel grants from Biogen Idec, Novartis, UCB, and the Guthy-Jackson Charitable Foundation and serves on scientific or educational advisory boards for UCB Pharma, Argenx, and Viela/Horizon; J. Bennett has received grant support from Mallinckrodt Pharmaceuticals, Novartis, Alexion, NIH, and Guthy Jackson Charitable Foundation; received consulting fees from Alexion, Horizon Therapeutics, Reistone-Bio, Mitsubishi Tanabe, Sanofi-Genzyme, Antigenomycs, Beigene, Genentech-Roche, TG Therapeutics, and Chugai; and provides services on Independent Data Safety Monitoring Boards for Clene Nanomedicine and Roche. He provides editorial assistance to the Journal of Neuro-Ophthalmology, MS Journal, Neurology: Neuroimmunology & Neuroinflammation, and Frontiers in Ophthalmology. In the past 36 months, B. Cree has received personal compensation for consulting from Alexion, Atara, Autobahn, Avotres, Biogen, EMD Serono, Gossamer Bio, Horizon, Neuron23, Novartis, Sanofi, TG Therapeutics, and Therini and received research support from Genentech; D. Rotstein has received research support from the MS Society of Canada, Consortium of MS Centers (CMSC), University of Toronto Division of Neurology, and Roche Canada. She has received speaker's or consultant's fees from Alexion, Biogen, EMD Serono, Novartis, Roche, and Sanofi-Genzyme; S. Pittock has received personal compensation for serving as a consultant for Genentech, Sage Therapeutics, Astellas, and UCB. He's received personal compensation for serving on scientific advisory boards or data safety monitoring boards for F. Hoffman-LaRoche AG, Genentech, and UCB. His institution has received compensation for serving as a consultant for Astellas, Alexion, and Viela Bio/MedImmune. All compensation is paid to Mayo Clinic. He has received research support from Alexion, Grifols, NIH, Viela Bio/MedImmune, F. Hoffman-LaRoche AG/Roche/Genentech, and NovelMed. All compensation is paid to Mayo Clinic. He has a patent, Patent# 8,889,102 (Application#12-678350, Neuromyelitis Optica Autoantibodies as a Marker for Neoplasia)—issued; a patent, Patent# 9,891,219B2 (Application#12-573942, Methods for Treating Neuromyelitis Optica (NMO) by Administration of Eculizumab to an individual that is Aquaporin-4 (AQP4)–IgG Autoantibody positive)—issued; Patents for Kelch11, LUZP4, Septin, MAP1b Abs, GFAP-IgG, and PDE10A pending. Go to Neurology.org/NN for full disclosures.

Figures

**Figure. Overview of NMOSD Delphi Consensus Process**
NMOSD = neuromyelitis optica spectrum disorder.

See this image and copyright information in PMC

References

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International Delphi Consensus on the Management of AQP4-IgG+ NMOSD: Recommendations for Eculizumab, Inebilizumab, and Satralizumab

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International Delphi Consensus on the Management of AQP4-IgG+ NMOSD: Recommendations for Eculizumab, Inebilizumab, and Satralizumab

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