Best practices in the use of human immunoglobulin preparations for intravenous administration in the treatment of rare neurological diseases

Aim. To describe best practices in using human normal immunoglobulin in patients with immune-mediated neurological disorders according to the data of one clinical center.

Materials and methods. From 2016 to 2021, 20 patients with various autoimmune disorders of the peripheral and central nervous system were treated with human normal immunoglobulin at the Neurology Unit No.1 of Pavlov First Saint Petersburg State Medical University. Treatment efficacy was assessed by changes in the neurological examination data according to specialized scales for specific diseases or clinical manifestations (INCAT, QMGS, MoCA, EDSS). Safety of the therapy was assessed considering the instructions to the drug.

Results. In the vast majority of patients, treatment allowed to stabilize the course of the disease or was accompanied by pronounced regression.

Conclusion. The considered clinical cases of the use of human normal immunoglobulin preparations demonstrate the possibility of their use in the treatment of a number of autoimmune neurological diseases for unregistered indications.

1. Середавкина Н.В., Решетняк Т.М., Насонов Е.Л. Место внутривенного иммуноглобулина при ревматических заболеваниях. Современная ревматология. 2015;9(4):59–67. DOI: 10.14412/1996-7012-2015-4-59-67.

2. Супонева Н.А., Пирадов М.А. Особенности проведения внутривенной иммунотерапии в неврологической клинике. Эффективная фармакотерапия. 2014;(58):12–23.

3. Lünemann J.D., Quast I., Dalakas M.C. Efficacy of intravenous immunoglobulin in neurological diseases. Neurotherapeutics. 2016;13(1):34–46. DOI: 10.1007/s13311-015-0391-5.

4. Никитин С.С., Борискина Л.М. Иммуноглобулины в неврологической практике: обзор литературы. Нервно-мышечные болезни. 2019;9(1):32–51. DOI: 10.17650/22228721-2019-9-1-32-51.

5. Souayah N., Hasan A., Khan H.M. et al. The safety profile of home infusion of intravenous immunoglobulin in patients with neuroimmunologic disorders. J. Clin. Neuromuscul. Dis. 2011;12(4):1–10. DOI: 10.1097/CND.0b013e3182212589.

6. Lehmann H.C., Burke D., Kuwabara S. Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immune pathogenesis and treatment. J. Neurol. Neurosurg. Psychiatry. 2019;90(9):981–987. DOI: 10.1136/jnnp-2019-320314.

7. Oaklander A.L., Lunn M.P.T., Hughes R.A.C. et al. Treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): an overview of systematic reviews. Cochrane Database Syst. Rev. 2017;1(1):CD010369. DOI: 10.1002/14651858.CD010369.pub2.

8. Van den Bergh P.Y., Hadden R.D., Bouche P. et al. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society – first revision. Eur. J. Neurol. 2010;17(3):356– 363. DOI: 10.1111/j.1468-1331.2009.02930.x.

9. Bukhari S., Bettin M., Cathro H.P. et al. Anti-neurofascin-associated nephrotic-range proteinuria in chronic inflammatory demyelinating polyneuropathy. Kidney Med. 2020;2(6):797–800. DOI: 10.1016/j.xkme.2020.06.016.

10. Chaudhry H.M., Mauermann M.L., Rajkumar S.V. Monoclonal gammopathy-associated peripheral neuropathy: Diagnosis and management. Mayo Clin. Proc. 2017;92(5):838–850. DOI: 10.1016/j.mayocp.2017.02.003.

11. Nobile-Orazio E., Bianco M., Nozza A. Advances in the treatment of paraproteinemic neuropathy. Curr. Treat. Options Neurol. 2017;19(12):43. DOI: 10.1007/s11940-017-0479-9.

12. Schmidt J. Current classification and management of inflammatory myopathies. J. Neuromuscul. Dis. 2018;5(2):109–129. DOI: 10.3233/JND-180308.

13. Barsotti S., Lundberg I.E. Current treatment for myositis. Curr. Treatm. Opt. Rheumatol. 2018;4(4):299–315. DOI: 10.1007/s40674-018-0106-2.

14. Joint Task Force of the EFNS and the PNS European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of multifocal motor neuropathy. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society – first revision. J. Peripher. Nerv. Syst. 2010;15(4):295–301. DOI: 10.1111/j.1529-8027.2010.00290.x.

15. Beadon K., Guimarães-Costa R., Léger J.M. Multifocal motor neuropathy. Curr. Opin. Neurol. 2018;31(5):559–564. DOI: 10.1097/WCO.0000000000000605.

16. Sarva H., Deik A., Ullah A. et al. Clinical Spectrum of Stiff Person Syndrome: A Review of Recent Reports. Tremor. Other Hyperkinet. Mov. (NY). 2016;6:340. DOI: 10.7916/ D85M65GD.

17. Lancaster E. The diagnosis and treatment of autoimmune encephalitis. J. Clin. Neurol. 2016;12(1):1–13. DOI: 10.3988/jcn.2016.12.1.1.

18. Achiron A., Kishner I., Dolev M. et al. Effect of intravenous immunoglobulin treatment on pregnancy and postpartum-related relapses in multiple sclerosis. J. Neurol. 2004;251(9):1133– 1137. DOI: 10.1007/s00415-004-0495-z.

19. Бисага Г.Н., Скулябин Д.И., Попов А.Е. Перспективы применения внутривенных иммуноглобулинов при рассеянном склерозе. Журнал неврологии и психиатрии им. С.С. Корсакова. Спецвыпуски. 2015;115(8-2):36–43. DOI: 10.17116/jnevro20151158236-43.

20. 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):1–16. DOI: 10.1007/s00415-0137169-7.