Consensus opinion on the management of patients with neuromyelitis optica spectrum diseases: issues of terminology and therapy
https://doi.org/10.14412/2074-2711-2022-6-139-148
Abstract
Neuromyelitis optica spectrum disorders (NMOSD) are a group of inflammatory diseases of the central nervous system characterized by episodes of immune-mediated astrocytopathy, inflammatory demyelination and axonal damage, predominantly affecting the optic nerves and spinal cord. Currently, there are several unresolved issues that make it difficult to provide medical care to patients with NMOSD. The objective of this publication is to present the results of the work of the Russian experts panel, held in September 2022. The consensus council of experts adopted the main definitions and terms for NMOSD, proposed a therapeutic algorithm to prevent exacerbations in patients with NMOSD with serum IgG antibodies to aquaporin-4 (AQP4-IgG).
The experts formulated profiles of patients with NMOSD to whom eculizumab may be recommended, and proposed a risk management plan during the treatment with this drug. The experts worked out the next steps, which are necessary for the improvement of medical care for these patients: development of unified electronic register of patients with NMOSD in the Russian Federation, development and approval of clinical guidelines.
About the Authors
V. S. KrasnovRussian Federation
Vladimir Sergeevich Krasnov
197022, St. Petersburg, Lev Tolstoy St., 6–8
Competing Interests:
The conflict of interest has not affected the results of the investigation.
K. Z. Bakhtiyarova
Russian Federation
450008, Ufa, Lenin St., 3
Competing Interests:
The conflict of interest has not affected the results of the investigation.
E. P. Evdoshenko
Russian Federation
197110, St. Petersburg, Dynamo Pr., 3
Competing Interests:
The conflict of interest has not affected the results of the investigation.
D. S. Korobko
Russian Federation
630087, Novosibirsk, Nemirovich-Danchenko St., 130
630091, Novosibirsk, Krasny Pr., 52
Competing Interests:
The conflict of interest has not affected the results of the investigation.
T. O. Simaniv
Russian Federation
125367, Moscow, Volokolamskoe Sh., 80
Competing Interests:
The conflict of interest has not affected the results of the investigation.
N. A. Totolyan
Russian Federation
197022, St. Petersburg, Lev Tolstoy St., 6–8
Competing Interests:
The conflict of interest has not affected the results of the investigation.
N. V. Khachanova
Russian Federation
117997, Moscow, Ostrovityanov St., 1
127015, Moscow, Pistcovaya St., 10
Competing Interests:
The conflict of interest has not affected the results of the investigation.
M. V. Shumilina
Russian Federation
197022, St. Petersburg, Lev Tolstoy St., 6–8
197110, St. Petersburg, Dynamo Pr., 3
Competing Interests:
The conflict of interest has not affected the results of the investigation.
M. V. Davydovskaya
Russian Federation
117997, Moscow, Ostrovityanov St., 1
143033, Moscow Region, Krasnogorsk, Karbyshev St., 4
Competing Interests:
The conflict of interest has not affected the results of the investigation.
References
1. Wingerchuk DM, Banwell B, Bennett JL, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015 Jul 14;85(2):177-89. doi:10.1212/WNL.0000000000001729
2. Wingerchuk DM, Lennon VA, Lucchinetti CF, et al. The spectrum of neuromyelitis optica. Lancet Neurol. 2007 Sep;6(9):805-15. doi:10.1016/S1474-4422(07)70216-8
3. Ma X, Kermode AG, Hu X, Qiu W. NMOSD acute attack: Understanding, treatment and innovative treatment prospect. J Neuroimmunol. 2020 Nov 15;348:577387. doi:10.1016/j.jneuroim.2020.577387
4. Starodubov VI, Zelenova OV, Abramov SI, et al. The first observational epidemiological study to determine the incidence and prevalence of disease range of neuromyelitis optica (Devik’s disease) on the territory of the Russian Federation. Sovremennye problem zdravookhraneniya i meditsinskoi statistiki = Current Problems of Health Care and Medical Statistics. 2021;(1):130-41. doi:10.24411/2312-2935-2021-00007 (In Russ.).
5. Weinshenker BG, Wingerchuk DM. Neuromyelitis spectrum disorders. Mayo Clin Proc. 2017 Apr;92(4):663-79. doi:10.1016/j.mayocp.2016.12.014
6. Whittam D, Wilson M, Hamid S, et al. What’s new in neuromyelitis optica? A short review for clinical neurologist. J Neurol. 2017 Nov;264(11):2330-4. doi:10.1007/s00415-017-8445-8
7. Lopez-Chiriboga AS, Majed M, Fryer J, et al. Association of MOG-IgG serostatus with relapse after acute disseminated encephalomyelitis and proposed diagnostic criteria for MOG-IgG-associated disorders. JAMA Neurol. 2018 Nov 1;75(11):1355-63. doi:10.1001/jamaneurol.2018.1814
8. Marignier R, Hacohen Y, Cobo-Calvo A, et al. Myelin-oligodendrocyte glycoprotein antibody-associated disease. Lancet Neurol. 2021 Sep;20(9):762-72. doi:10.1016/S1474-4422(21)00218-0
9. Bruscolini A, Sacchetti M, La Cava M, et al. Diagnosis and management of neuromyelitis optica spectrum disorders – An update. Autoimmun Rev. 2018 Mar;17(3):195-200. doi:10.1016/j.autrev.2018.01.001
10. Belova AN, Boyko AN, Belova EM. Diagnostic criteria for neuromyelitisoptica spectrum disorders. Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova = S.S. Korsakov Journal of Neurology and Psychiatry. 2016;116(2-2):32-40. doi:10.17116/jnevro20161162232-40 (In Russ.).
11. Vinogradova ES, Novikov PI, Moiseev SV. Neuromyelitics optica (Devic’s syndrome) is a rare variant of the nervous system impairment in systemic lupus erythematosus. Russkii meditsinskii zhurnal = Russian Medical Journal. 2018;12(2):103-6 (In Russ.).
12. Tokareva YuV, Kotov AS, Panteleeva MV, Bunak MS. Neuromyelitis optica spectrum disorders in pediatric patients. Nevrologiya, neiropsikhiatriya, psikhosomatika = Neurology, Neuropsychiatry, Psychosomatics. 2018;10(1):60-4. doi:10.14412/2074-2711-2018-1-60-64 Russ.).
13. Simaniv TO, Vasil’ev AV, Askarova LSh, Zakharova MN. Neuromyelitis optica and neuromyelitis optica spectrum disorders. Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova = S.S. Korsakov Journal of Neurology and Psychiatry. 2019;119(10-2):35-48. doi:10.17116/jnevro20191191035 (In Russ.).
14. Krasnov VS, Totolyan NA, Nazarov VD, et al. Actual issues of serum aquaporin-4 autoantibodies evaluation in the diagnostics of neuromyelitis optica spectrum disorders. Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova = S.S. Korsakov Journal of Neurology and Psychiatry. 2020;120(7-2):24-31. doi:10.17116/jnevro202012007224 (In Russ.).
15. Krasnov VS, Makshakov GS, Kalinin IV, et al. Comorbidity of neuromyelitis optica spectrum disorder and systemic lupus erythematosus. Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova = S.S. Korsakov Journal of Neurology and Psychiatry. 2020;120(7):102-8. doi:10.17116/jnevro2020120071102 (In Russ.).
16. Boyko AN, Bakhtiyarova KZ, Brylev LV, et al. Consensus of expert advices on routing, diagnosis, and management of patients with neuromyelitis optica spectrum disorders. Nevrologiya, neiropsikhiatriya, psikhosomatika = Neurology, Neuropsychiatry, Psychosomatics. 2021;13(5):140-7. doi:10.14412/2074-2711-2021-5-140-147 (In Russ.).
17. Hemmer B. Diagnose und Therapie der Multiplen Sklerose, Neuromyelitis-opticaSpektrum-Erkrankungen und MOG-IgGassoziierten Erkrankungen, S2k-Leitlinie, 2021, in: Deutsche Gesellschaft für Neurologie (Hrsg.), Leitlinien für Diagnostik und Therapie in der Neurologie. Available from: www.dgn.org/leitlinien
18. Shosha E, Aljarallah SA, Fugham NA, et al. Saudi consensus recommendations on the management of neuromyelitis optica spectrum disorders (NMOSD). Mult Scler Relat Disord. 2022 Oct;66:104062. doi:10.1016/j.msard.2022.104062
19. Davydovskaya MV, Khachanova NV, Klabukova DL. Practical aspects of the differential diagnosis of primary progressive multiple sclerosis. Klinicheskaya farmakologiya i terapiya = Clinical Pharmacology and Therapy. 2018;27(5):69-75. doi:10.32756/0869-5490-2018-5-69-75 (In Russ.).
20. Davydovskaya MV. Neuromyelitis optica spectrum disorder: importance and difficulties of differential diagnosis with multiple sclerosis. Lechaschiy vrach. 2021;(5):62-3. doi:10.51793/OS.2021.95.91.013 (In Russ.).
21. Simaniv TO, Kochergin IA, Zakharova MN, et al. Clinical and epidemiological aspects of neuromyelitis optic spectrum diseases in the Russian population. Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova = S.S. Korsakov Journal of Neurology and Psychiatry. 2021;121(7):96-103. doi:10.17116/jnevro202112107196 (In Russ.).
22. Krasnov VS, Prakhova LN, Totolyan NA. Current view on the diagnosis and treatment of neuromyelitis optica spectrum disorders exacerbations. Nevrologiya, neiropsikhiatriya, psikhosomatika = Neurology, Neuropsychiatry, Psychosomatics. 2022;14(5):69-76. doi:10.14412/2074-2711-2022-5-69-76 (In Russ.).
23. Pittock SJ, Berthele A, Fujihara K, et al. Eculizumab in Aquaporin-4–Positive Neuromyelitis Optica Spectrum Disorder. N Engl J Med. 2019 Aug 15;381(7):614-25. doi:10.1056/NEJMoa1900866
24. Palace J, Wingerchuk DM, Fujihara K, et al. Benefits of eculizumab in AQP4+ neuromyelitis optica spectrum disorder: Subgroup analyses of the randomized controlled phase 3 PREVENT trial. Mult Scler Relat Disord. 2021 Jan;47:102641. doi:10.1016/j.msard.2020.102641
25. Cree BA, Bennett JL, Kim HJ, et al. Inebilizumab for the treatment of neuromyelitis optica spectrum disorder (N-MOmentum): a double-blind, randomised placebo-controlled phase 2/3 trial. Lancet. 2019 Oct 12;394(10206):1352-63. doi:10.1016/S0140-6736(19)31817-3
26. Prakhova LN, Krasnov VS, Kasatkin DS, Korobko DS. Local experience of IL-6 pathway inhibition with satralizumab for patients with neuromyelitis optica spectrum disorder. Differential diagnosis of multiple sclerosis in children. Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova = S.S. Korsakov Journal of Neurology and Psychiatry. 2022;122(7-2):68-72. doi:10.17116/jnevro202212207268 (In Russ.).
27. Contentti EC, Rojas JI, Cristiano E, et al. Latin American consensus recommendations for management and treatment of neuromyelitis optica spectrum disorders in clinical practice. Mult Scler Relat Disord. 2020 Oct; 45:102428. doi:10.1016/j.msard.2020.102428
28. Zhang W, Cui L, Zhang Y, et al. Questioning the existence of monophasic neuromyelitis optica spectrum disorder by defining a novel long-term relapse-free form from a large Chinese population. J Neurol. 2020 Apr;267(4):1197-205. doi:10.1007/s00415-019-09685-3
29. Kim SH, Jang H, Park NY, et al. Discontinuation of immunosuppressive therapy in patients with neuromyelitis optica spectrum disorder with aquaporin-4 antibodies. Neurol Neuroimmunol Neuroinflamm. 2021 Feb 23;8(2):e947. doi:10.1212/NXI.0000000000000947
30. Palace J, Lin DY, Zeng D, et al. Outcome prediction models in AQP4-IgG positive neuromyelitis optica spectrum disorders. Brain. 2019 May 1;142(5):1310-23. doi:10.1093/brain/awz054
31. Hu H, You X, Ye J. Short transverse myelitis in Chinese patients with neuromyelitis optica spectrum disorders. Mult Scler Relat Disord. 2018 Apr;21:78-83. doi:10.1016/j.msard.2018.02.022
32. Li Y, Zhang J, Zhou Y, et al. Analysis of Predictive Risk Factors in Aquaporin-4-IgG Positive Highly Active Neuromyelitis Optica Spectrum Disorders. Front Neurol. 2021 Aug 26;12:731835. doi:10.3389/fneur.2021.731835. eCollection 2021.
33. Zhang C, Zhang M, Qiu W, et al. Safety and efficacy of tocilizumab versus azathioprine in highly relapsing neuromyelitis optica spectrum disorder (TANGO): an open-label, multicentre, randomised, phase 2 trial. Lancet Neurol. 2020 May;19(5):391-401. doi:10.1016/S1474-4422(20)30070-3
34. Ringelstein M, Ayzenberg I, Harmel J, et al. Long-term Therapy With Interleukin 6 Receptor Blockade in Highly Active Neuromyelitis Optica Spectrum Disorder. JAMA Neurol. 2015 Jul;72(7):756-63. doi:10.1001/jamaneurol.2015.0533
35. Kleiter I, Gahlen A, Borisow N, et al. Apheresis therapies for NMOSD attacks: A retrospective study of 207 therapeutic interventions. Neurol Neuroimmunol Neuroinflamm. 2018 Sep 26;5(6):e504. doi:10.1212/NXI.0000000000000504
36. Bizzoco E, Lolli F, Repice AM, et al. Prevalence of neuromyelitis optica spectrum disorder and phenotype distribution. J Neurol. 2009 Nov;256(11):1891-8. doi:10.1007/s00415-009-5171-x
37. Yamamura T, Kleiter I, Fujihara K, et al. Trial of satralizumab in neuromyelitis optica spectrum disorder. N Engl J Med. 2019 Nov 28;381(22):2114-24. doi:10.1056/NEJMoa1901747
38. Tramboulsee A, Greenberg BM, Bennett JL, et al. Safety and efficacy of satralizumab monotherapy in neuromyelitis optica spectrum disoder: a randomised, double-blind, multicentre, placebo-conroled phase 3 trial. Lancet Neurol. 2020 May;19(5):402-12. doi:10.1016/S1474-4422(20)30078-8
39. Shosha E, Dubey D, Palace J, et al. Area postrema syndrome: Frequency, criteria, and severity in AQP4-IgG–positive NMOSD. Neurology. 2018 Oct 23;91(17):e1642-e1651. doi:10.1212/WNL.0000000000006392
40. Kurtzke JF. Rating neurologic impairment in multiple sclerosis: An Expanded Disability Status Scale (EDSS). Neurology. 1983;33(11):1444-52. doi:10.1212/wnl.33.11.1444
41. Schwartz СЕ, Stark RB, Stucky BD. Response-shift effects in neuromyelitis optica spectrum disorder: a secondary analysis of clinical trial data. Qual Life Res. 2021 May;30(5):1267-82. doi:10.1007/s11136-020-02707-y.56
42. Songthammawat T, Srisupa-Olan T, Siritho S, et al. A pilot study comparing treatments for severe attacks of neuromyelitis optica spectrum disorders: intravenous methylprednisolone (IVMP) with add-on plasma exchange (PLEX) versus simultaneous ivmp and PLEX. Mult Scler Relat Disord. 2020 Feb; 38:101506. doi:10.1016/j.msard.2019.101506
43. Li X, Tian D-C, Fan M, et al. Intravenous immunoglobulin for acute attacks in neuromyelitis optica spectrum disorders (NMOSD). Mult Scler Relat Disord. 2020 Sep;44: 102325. doi:10.1016/j.msard.2020.102325.55
44. Sellner J, Boggild M, Clanet M, et al. EFNS guidelines on diagnosis and management of neuromyelitis optica. Eur J Neurol. 2010 Aug;17(8):1019-32. doi:10.1111/j.1468-1331.2010.03066.x
45. Shi Z, Du Q, Chen H, et al. Effects of immunotherapies and prognostic predictors in neuromyelitis optica spectrum disorder: a prospective cohort study. J Neurol. 2020 Apr; 267(4):913-24. doi:10.1007/s00415-019-09649-7
46. Regulatory workshop on clinical trials designs in neuromyelitis optica spectrum disorders (NMOSD). Report of EMA workshop 10 October 2014. Available from: https://www.ema.europa.eu/en/events/regulatory-workshop-clinical-trials-designs-neuromyelitis-optica-spectrum-disorders
47. Levy M, Fujihara K, Palace J. New therapies for neuromyelitis optica spectrum disorder. Lancet Neurol. 2021 Jan;20(1):60-7. doi:10.1016/S1474-4422(20)30392-6
48. Ciron J, Audoin B, Bourre B, et al. Recommendations for the use of Rituximab in neuromyelitis optica spectrum disorders. Rev Neurol (Paris). 2018 Apr;174(4):255-64. doi:10.1016/j.neurol.2017.11.005
49. Gao F, Chai B, Gu C, et al. Effectiveness of rituximab in neuromyelitis optica: a metaanalysis. BMC Neurol. 2019 Mar 6;19(1):36. doi:10.1186/s12883-019-1261-2
50. Mealy MA, Wingerchuk DM, Palace J, et al. Comparison of relapse and treatment failure rates among patients with neuromyelitis optica: multicenter study of treatment efficacy. JAMA Neurol. 2014 Mar;71(3):324-30. doi:10.1001/jamaneurol.2013.5699
51. Nikoo Z, Badihian S, Shaygannejad V, et al. Comparison of the efficacy of azathioprine and rituximab in neuromyelitis optica spectrum disorder: a randomized clinical trial. J Neurol. 2017 Sep;264(9):2003-9. doi:10.1007/s00415-017-8590-0. Epub 2017 Aug 22.
52. Songwisit S, Kosiyakul P, Jitprapaikulsan J, et al. Efficacy and safety of mycophenolate mofetil therapy in neuromyelitis optica spectrum disorders: a systematic review and metaanalysis. Sci Rep. 2020 Oct 7;10(1):16727. doi:10.1038/s41598-020-73882-8
53. Kimbrough DJ, Fujihara K, Jacob A, et al. Treatment of neuromyelitis optica: Review and recommendations. Mult Scler Relat Disord. 2012 Oct;1(4):180-7. doi:10.1016/j.msard.2012.06.00245
54. Trebst C, Jarius S, Berthele A, et al. Update on the diagnosis and treatment of neuromyelitis optica: Recommendations of the Neuromyelitis Optica Study Group (NEMOS). J Neurol. 2014;261:1-16. doi:10.1007/s00415-013-7169-7
55. Wingerchuk DM, Fujihara K, Palace J, et al. Long-Term Safety and Efficacy of Eculizumab in Aquaporin-4 IgG-Positive NMOSD. Ann Neurol. 2021 Jun;89(6):1088-98. doi:10.1002/ana.26049
56. Pittock SJ, Fujihara K, Palace J, et al. Eculizumab monotherapy for NMOSD: Data from PREVENT and its open-label extension. Mult Scler J. 2022;28(3):480-6. doi:10.1177/13524585211038291
57. Wingerchuk DM, Zhang I, Kielhorn A, et al. Network meta-analysis of Food and Drug Administration-approved treatment options for adults with aquaporin-4 immunoglobulin Gpositive neuromyelitis optica spectrum disorder. Neurol Ther. 2022 Mar;11(1):123-35. doi:10.1007/s40120-021-00295-8
58. Article E. Resolution of the interdisciplinary council experts for prevention of severe infections in patients with genetic disorders of regulation of the complement system, receiving therapy with eculizumab. Epidemiologiya i vaktsinoprofilaktika = Epidemiology and Vaccinal Prevention. 2017;16(1):51-4 (In Russ.).
59. Velasco M, Zarco LA, Agudelo-Arrieta M. Effectiveness of treatments in Neuromyelitis optica to modify the course of disease in adult patients. Systematic review of literature. Mult Scler Relat Disord. 2021 May;50:102869. doi:10.1016/j.msard.2021.102869
Review
For citations:
Krasnov VS, Bakhtiyarova KZ, Evdoshenko EP, Korobko DS, Simaniv TO, Totolyan NA, Khachanova NV, Shumilina MV, Davydovskaya MV. Consensus opinion on the management of patients with neuromyelitis optica spectrum diseases: issues of terminology and therapy. Nevrologiya, neiropsikhiatriya, psikhosomatika = Neurology, Neuropsychiatry, Psychosomatics. 2022;14(6):139-148. (In Russ.) https://doi.org/10.14412/2074-2711-2022-6-139-148