Aggressive multiple sclerosis in the Republic of Bashkortostan

Treatment of patients with aggressive multiple sclerosis (MS) characterized by severe progression of disability within a short period of time is a complex task as there are no uniform criteria for determining such disease progression type and, accordingly, no optimal strategies for prescribing medication. Standardised criteria are also needed to assess prevalence in the general patient population and to plan medical and social care.

Objective: to analyse clinical and demographic characteristics of patients with aggressive MS in the Republic of Bashkortostan (RB).

Material and methods. The study included 2670 patients registered in the Multiple Sclerosis Centre of the Republic of Bashkortostan. To determine the types of MS progression, criteria of the 2022 clinical guidelines for MS were used. When analysing the data on disability, the data from the control group, which consisted of MS patients without limitations in working capacity and disability, were also used.

Results. Rapidly progressive MS (RPMS) and highly active MS (HAMS) were diagnosed in 8.9% of the total number of patients. Women predominated in both groups. In the group of patients with aggressive MS, the progression rate was significantly higher than in the HAMS group. Six percent of patients included in the registry became disabled (group II or I) within 5 years of the disease, and 10% within 10 years. These groups are characterized by a high rate of disease progression.

Conclusion. In the RB, RPMS and HAMS were diagnosed in 8.9% of patients, with a predominance of women.

1. Koch-Henriksen N, Magyari M. Apparent changes in the epidemiology and severity of multiple sclerosis. Nat Rev Neurol. 2021 Nov;17(11):676-88. doi: 10.1038/s41582-021-00556-y. Epub 2021 Sep 28.

2. Clinical guidelines – Multiple sclerosis – 2022–2023–2024 (13.07.2022). Approved by the Ministry of Health of the Russian Federation (In Russ.).

3. Correale J, Rush CA, Barboza A. Are highly active and aggressive multiple sclerosis the same entity? Front Neurol. 2023 Mar 3;14:1132170. doi: 10.3389/fneur.2023.1132170

4. Thompson AJ, Banwell BL, Barkhof F, et al. Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria. Lancet Neurol. 2018 Feb;17(2):162-73. doi: 10.1016/S1474-4422(17)30470-2. Epub 2017 Dec 21.

5. Lublin FD, Reingold SC, Cohen JA, et al. Defining the clinical course of multiple sclerosis: the 2013 revisions. Neurology. 2014 Jul 15;83(3):278-86. doi: 10.1212/WNL.0000000000000560. Epub 2014 May 28.

6. Iacobaeus E, Arrambide G, Amato MP, et al. Aggressive multiple sclerosis (1): toward a definition of the phenotype. Mult Scler. 2020;26:1031- 44. doi: 10.1177/1352458520925369

7. Menon S, Shirani A, Zhao Y, et al. Characterising aggressive multiple sclerosis. J Neurol Neurosurg Psychiatry. 2013 Nov;84(11):1192-8. doi: 10.1136/jnnp-2013-304951. Epub 2013 Jun 6.

8. Ellenberger D, Flachenecker P, Fneish F, et al. Aggressive multiple sclerosis: a matter of measurement and timing. Brain. 2020 Dec 5;143(11):e97. doi: 10.1093/brain/awaa306

9. Gholipour T, Healy B, Baruch NF, et al. Demographic and clinical characteristics of malignant multiple sclerosis. Neurology. 2011 Jun 7;76(23):1996-2001. doi: 10.1212/WNL.0b013e31821e559d

10. Freedman MS, Rush CA. Severe, Highly Active, or Aggressive Multiple Sclerosis. Continuum (Minneap Minn). 2016 Jun;22(3):761- 84. doi: 10.1212/CON.0000000000000331. Erratum in: Continuum (Minneap Minn). 2018 Jun;24(3, BEHAVIORAL NEUROLOGY AND PSYCHIATRY):967. doi: 10.1212/01.CON.0000534994.77727.73

11. Tintore M, Arrambide G, Otero-Romero S, et al. The long-term outcomes of CIS patients in the Barcelona inception cohort: Looking back to recognize aggressive MS. Mult Scler. 2020 Nov;26(13):1658-69. doi: 10.1177/1352458519877810. Epub 2019 Oct 15.

12. Malpas CB, Manouchehrinia A, Sharmin S, et al. Early clinical markers of aggressive multiple sclerosis. Brain. 2020 May 1;143(5):1400- 1413. doi: 10.1093/brain/awaa081

13. Rush CA, MacLean HJ, Freedman MS. Aggressive multiple sclerosis: proposed definition and treatment algorithm. Nat Rev Neurol. 2015 Jul;11(7):379-89. doi: 10.1038/nrneurol.2015.85. Epub 2015 Jun 2.

14. CHMP. Committee for Medicinal Products for Human Use (CHMP) Assessment Report; 2014. Available at: www.ema.europa.eu (accessed 13.04.2020).

15. Huisman E, Papadimitropoulou K, Jarrett J, et al. Systematic literature review and network meta-analysis in highly active relapsing-remitting multiple sclerosis and rapidly evolving severe multiple sclerosis. BMJ Open. 2017 Mar 10;7(3):e013430. doi: 10.1136/bmjopen-2016-013430

16. Zakharova MN. Monoclonal antibodies in the treatment of neurological diseases: current state and future development. Annaly klinicheskoj i eksperimental’noj nevrologii = Annals of Сlinical and Experimental Neurology. 2018;12(5S):99- 104. doi: 10.25692/ACEN.2018.5.13 (In Russ.).

17. Diaz C, Zarco LA, Rivera DM. Highly active multiple sclerosis: An update. Mult Scler Relat Disord. 2019 May;30:215-24. doi: 10.1016/j.msard.2019.01.039. Epub 2019 Jan 24.

18. Khachanova NV. Highly active multiple sclerosis: options for monoclonal antibody therapy. Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova = S.S. Korsakov Journal of Neurology and Psychiatry. 2019;119(10-2):49-57. doi: 10.17116/jnevro201911910249 (In Russ.).

19. Schmidt TE, Gerasimova AE. Aggressive multiple sclerosis. Nevrologicheskiy zhurnal = Neurological Journal. 2011;16(5):4-10 (In Russ.).

20. Fink K. Multiple sclerosis e-registries. Neurodegener Dis Manag. 2016 Dec;6(6s):23- 25. doi: 10.2217/nmt-2016-0051

21. Bebo BF Jr, Fox RJ, Lee K, et al. Landscape of MS patient cohorts and registries: Recommendations for maximizing impact. Mult Scler. 2018 Apr;24(5):579-86. doi: 10.1177/1352458517698250. Epub 2017 Mar 1.

22. Schmidt TE, Yakhno NN. Multiple sclerosis: a guide for doctors: [Inflammation. Degeneration]. 3rd ed. Moscow: MEDpressinform; 2012. 271 p. (In Russ.).

23. Gumerova ZB, Safronova EV. An analysis of disability in consequence of a multiple sclerosis in the Republic of Bashkortostan for a period of 2014–2016 years. Medico-sotsialnye problemy invalidnosty. 2018;(1):89-91 (In Russ.).

24. Kochergin IA, Abramova AA, Zakharova MN. Experience in designing an electronic multiple sclerosis registry. Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova = S.S. Korsakov Journal of Neurology and Psychiatry. 2020;120(7-2):77-82. doi: 10.17116/jnevro202012007277 (In Russ.).

25. Kern DM, Cepeda MS. Treatment patterns and comorbid burden of patients newly diagnosed with multiple sclerosis in the United States. BMC Neurol. 2020 Aug 11;20(1):296. doi: 10.1186/s12883-020-01882-2

26. Confavreux C, Vukusic S, Adeleine P. Early clinical predictors and progression of irreversible disability in multiple sclerosis: an amnesic process. Brain. 2003 Apr;126(Pt 4): 770-82. doi: 10.1093/brain/awg081

27. Zivadinov R, Cookfair DL, Krupp L, et al. Factors associated with benign multiple sclerosis in the New York State MS Consortium (NYSMSC). BMC Neurol. 2016 Jul 15;16:102. doi: 10.1186/s12883-016-0623-2

28. Traboulsee AL, Cornelisse P, Sandberg-Wollheim M, et al. Prognostic factors for long-term outcomes in relapsing-remitting multiple sclerosis. Mult Scler J Exp Transl Clin. 2016 Sep 6;2:2055217316666406. doi: 10.1177/2055217316666406

29. Simone IL, Carrara D, Tortorella C, et al. Course and prognosis in early-onset MS: comparison with adult-onset forms. Neurology. 2002 Dec 24;59(12):1922-8. doi: 10.1212/01.wnl.0000036907.37650.8e

30. Ness JM, Chabas D, Sadovnick AD, et al; International Pediatric MS Study Group. Clinical features of children and adolescents with multiple sclerosis. Neurology. 2007 Apr 17;68(16 Suppl 2):S37-45. doi: 10.1212/01.wnl.0000259447.77476.a9

31. Boyko OV, Khoroshylova II, Petrov SV, et al. Changes in the quality of life in patients with multiple sclerosis treated with ocrelizumab. Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova = S.S. Korsakov Journal of Neurology and Psychiatry. 2019;119(10-2):120-7. doi: 10.17116/jnevro201911910120 (In Russ.).

32. Boyko AN, Bakhtiyarova KZ, Sherman MA, et al. Results of a study of the quality of life in patients with highly active multiple sclerosis in Russia. Nevrologiya, neiropsikhiatriya, psikhosomatika = Neurology, Neuropsychiatry, Psychosomatics. 2022;14(Suppl 1):9-15. doi: 10.14412/2074-2711-2022-1S-9-15 (In Russ.).

33. Ruggieri S, Pontecorvo S, Tortorella C, et al. Induction treatment strategy in multiple sclerosis: a review of past experience and future perspectives. Mult Scler Demyelinat Disord. 2018;3:5-9.