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Review
. 2024 Jan;271(1):141-176.
doi: 10.1007/s00415-023-11910-z. Epub 2023 Sep 7.

Update on the diagnosis and treatment of neuromyelitis optica spectrum disorders (NMOSD) - revised recommendations of the Neuromyelitis Optica Study Group (NEMOS). Part II: Attack therapy and long-term management

Tania Kümpfel  1 , Katrin Giglhuber  2 , Orhan Aktas  3 , Ilya Ayzenberg  4 , Judith Bellmann-Strobl  5   6   7   8 , Vivien Häußler  9 , Joachim Havla  10 , Kerstin Hellwig  4 , Martin W Hümmert  11 , Sven Jarius  12 , Ingo Kleiter  4   13 , Luisa Klotz  14 , Markus Krumbholz  15   16   17 , Friedemann Paul  5   6   7   8 , Marius Ringelstein  3   18 , Klemens Ruprecht  5 , Makbule Senel  19 , Jan-Patrick Stellmann  9   20   21 , Florian Then Bergh  22 , Corinna Trebst  12 , Hayrettin Tumani  20 , Clemens Warnke  23 , Brigitte Wildemann  13 , Achim Berthele  24 ; Neuromyelitis Optica Study Group (NEMOS)
Collaborators, Affiliations
Review

Update on the diagnosis and treatment of neuromyelitis optica spectrum disorders (NMOSD) - revised recommendations of the Neuromyelitis Optica Study Group (NEMOS). Part II: Attack therapy and long-term management

Tania Kümpfel et al. J Neurol. 2024 Jan.

Erratum in

Abstract

This manuscript presents practical recommendations for managing acute attacks and implementing preventive immunotherapies for neuromyelitis optica spectrum disorders (NMOSD), a rare autoimmune disease that causes severe inflammation in the central nervous system (CNS), primarily affecting the optic nerves, spinal cord, and brainstem. The pillars of NMOSD therapy are attack treatment and attack prevention to minimize the accrual of neurological disability. Aquaporin-4 immunoglobulin G antibodies (AQP4-IgG) are a diagnostic marker of the disease and play a significant role in its pathogenicity. Recent advances in understanding NMOSD have led to the development of new therapies and the completion of randomized controlled trials. Four preventive immunotherapies have now been approved for AQP4-IgG-positive NMOSD in many regions of the world: eculizumab, ravulizumab - most recently-, inebilizumab, and satralizumab. These new drugs may potentially substitute rituximab and classical immunosuppressive therapies, which were as yet the mainstay of treatment for both, AQP4-IgG-positive and -negative NMOSD. Here, the Neuromyelitis Optica Study Group (NEMOS) provides an overview of the current state of knowledge on NMOSD treatments and offers statements and practical recommendations on the therapy management and use of all available immunotherapies for this disease. Unmet needs and AQP4-IgG-negative NMOSD are also discussed. The recommendations were developed using a Delphi-based consensus method among the core author group and at expert discussions at NEMOS meetings.

Keywords: Aquaporin-4 (AQP4); Attacks; Disability; Double-negative NMOSD; Immunotherapies; Long-term management; Neuromyelitis optica spectrum disorders (NMOSD).

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Conflict of interest statement

TK has received speaker honoraria and/or personal fees for advisory boards from Roche Pharma, Alexion/Astra Zeneca, Horizon, Merck, Chugai and Biogen. The Institution she works for has received grant support for her research from Bayer-Schering AG, Novartis and Chugai Pharma in the past. None resulted in a conflict of interest. KG has received travel support from UCB. OA has received speaking honoraria and travel grants from Alexion, Almirall, Biogen, Celgene, Merck, Novartis, Roche, and VielaBio. IA has received speaking honoraria and scientific advisory board compensation from Alexion, Roche, Merck Serono, Santhera, and Sanofi Genzyme and research support from Chugai and Diamed. JBS has received speaking honoraria and travel grants from Bayer Healthcare, sanofiaventis/Genzyme, and Biogen and compensation for serving on a scientific advisory board from Roche. VH reports no conflicts of interest. JH reports a grant for OCT research from the Friedrich-Baur-Stiftung, personal fees and nonfinancial support from Merck, Alexion, Novartis, Roche, Santhera, Biogen, Heidelberg Engineering, and Sanofi Genzyme, and non-financial support from the Guthy-Jackson Charitable Foundation; JH is partially funded by the German Federal Ministry of Education and Research [grant numbers 01ZZ1603[A-D] and 01ZZ1804[A-H] (DIFUTURE)]. KH reports no conflicts of interest. MH reports no conflicts of interest. SJ reports no conflicts of interest. IK has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities from Alexion, Almirall, Bayer, Biogen, Hexal, Horizon, Merck, Neuraxpharm, Sanofi, and Roche/Chugai. LK has received compensation for serving on scientific advisory boards from Alexion, Biogen, BMS, Celgene GmbH, Genzyme, Horizon, Janssen, Merck Serono, Novartis, and Roche. She has received speaker honoraria and travel support from Bayer, Biogen, Celgene GmbH, Genzyme, Grifols, Merck Serono, Novartis, Roche, Santhera, and Teva. She receives research support from the German Research Foundation, the IZKF Münster, IMF Münster, Biogen, Immunic AG, Novartis, and Merck Serono. MK has received travel funding, speaker honoraria, and research support from Bristol Myers Squibb, Merck, Novartis, and Roche. FP has received grants from the Guthy Jackson Charitable Foundation, German Research Foundation, German Federal Ministry of Education and Research, Novartis, Bayer, Novartis, Biogen Idec, Teva, Alexion, Roche, Horizon, Sanofi-Aventis/Genzyme, Merck Serono, Chugai, German Research Council, Werth Stiftung of the City of Cologne, Arthur Arnstein Stiftung Berlin, EU FP7 Framework Program, National Multiple Sclerosis (USA), MedImmune, Shire, and Alexion, and has a patent on Foveal Morphometry pending to Nocturne GmbH; he is Academic Editor at PLoS ONE and Associate Editor of Neurology® Neuroimmunology & Neuroinflammation. MR has received speaker honoraria from Novartis, Bayer Vital GmbH, Roche, Alexion, and Ipsen and travel reimbursement from Bayer Schering, Biogen Idec, Merz, Genzyme, Teva, Roche, and Merck. KR received research support from Novartis Pharma, Merck Serono, German Ministry of Education and Research, European Union (821283–2), Stiftung Charité (BIH Clinical Fellow Program) and Arthur Arnstein Foundation, and received speaker honoraria from Bayer and travel grants from Guthy Jackson Charitable Foundation. MS has received consulting and/or speaker honoraria from Alexion, Bayer, Biogen, Bristol-Myers-Squibb, Merck, Roche, and Sanofi Genzyme. She has received research funding from the Hertha-Nathorff-Program. PS reports no conflict of interest. FTB has received personal compensation for advisory board participation or speaking, and through his institution has received research support for investigator-initiated studies or for attending scientific meetings, from Actelion, Alexion, Bayer-Schering, Biogen, Merck, Novartis, Roche, Sanofi-Genzyme, Teva, UCB, the German Research Foundation (DFG), and the German Federal Ministry of Education and Research (BMBF). CT has received honoraria for consultation and expert testimony from Alexion Pharma Germany GmbH, Chugai Pharma Germany GmbH, and Roche Pharma GmbH HT received research institutional support and/or consulting/speaker honoraria from Alexion, Bayer, Biogen, Bristol-Myers Squibb, Celgene, Diamed, Fresenius, Fujirebio, GlaxoSmithKline, Horizon, Janssen-Cilag, Merck, Novartis, Roche, Sanofi Genzyme, TEVA. His research is also funded by Ministry of Education and Research (BMBF), Ministry of Science, Research and Arts Baden Württemberg (MWK-BW), German Society of Multiple Sclerosis (DMSG), DMS-Stiftung, AMSEL-Stiftung, Bayern-DMSG and Chemische Fabrik Karl Bucher GmbH BW has received grants from the German Ministry of Education and Research, Deutsche Forschungsgemeinschaft, Dietmar Hopp Stiftung, Klaus Tschira Stiftung, and Merck, and personal fees from Alexion, Bayer, Biogen, Roche. CW has received institutional support from Novartis, Alexion, Sanofi Genzyme, Biogen, Merck, and Roche AB has received consulting and/or speaking fees from Alexion, Bayer Healthcare, Biogen, Celgene, Horizon and Roche. His institution has received compensations for clinical trials from Alexion, Biogen, Merck, Novartis, Roche, and Sanofi.

Figures

Fig. 1
Fig. 1
Attack therapy
Fig. 2
Fig. 2
Long-term therapy for AQP4-IgG-positive NMOSD
Fig. 3
Fig. 3
Long-term therapy for double-negative NMOSD
See this image and copyright information in PMC

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