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About 15–20% of myasthenic patients are refractory to standard steroid therapy. The present study was aimed at evaluation of efficacy and analysis of the algorithm of use of a novel immunosuppressant of natural origin, cyclosporine A, in the treatment of severe refractory forms of myasthenia gravis. In this work, for the first time on a large group of myasthenic patients (51 patients who became ill at 5–80 years of age), mechanisms of action of cyclosporine (sandimmun orally 2.5–5 mg/kg) on main levels of neuro-muscular junction were studied, and new confirmation of selective immune-modulating activity of cyclosporine (without total decrease of patient’s immune system) was presented. High efficacy and good tolerability of the drug in different pathogenic subtypes of myasthenia gravis were shown. Cyclosporin may be recommended in refractory steroid-dependent myasthenia, as well as in cases of combination of myasthenia with thymoma, autoimmune disorders and immunodeficiency viral infections.
The role of cyclosporin in the treatment of severe myasthenia: a clinical study of 51 patients
- Authors: Shcherbakova N.I.1, Pavlova E.М.2, Lantsova V.B.1, Savitskaya N.G.1, Pavlov E.V.1, Retinskaya I.G.1, Zakutnaya V.N.1
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Affiliations:
- Research Center of Neurology, Russian Academy of Medical Sciences
- The A.I. Burnazian Federal Medical Biophysical Center, Federal Medical Biological Agency
- Issue: Vol 7, No 2 (2013)
- Pages: 4-10
- Section: Original articles
- URL: https://annaly-nevrologii.com/journal/pathID/article/view/242
- DOI: https://doi.org/10.17816/psaic242
- ID: 242
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About the authors
N. I. Shcherbakova
Research Center of Neurology, Russian Academy of Medical Sciences
Author for correspondence.
Email: shnata@inbox.ru
Russian Federation, Moscow
E. М. Pavlova
The A.I. Burnazian Federal Medical Biophysical Center, Federal Medical Biological Agency
Email: shnata@inbox.ru
Russian Federation, Moscow
V. B. Lantsova
Research Center of Neurology, Russian Academy of Medical Sciences
Email: shnata@inbox.ru
Russian Federation, Moscow
N. G. Savitskaya
Research Center of Neurology, Russian Academy of Medical Sciences
Email: shnata@inbox.ru
Russian Federation, Moscow
E. V. Pavlov
Research Center of Neurology, Russian Academy of Medical Sciences
Email: shnata@inbox.ru
Russian Federation, Moscow
I. G. Retinskaya
Research Center of Neurology, Russian Academy of Medical Sciences
Email: shnata@inbox.ru
Russian Federation, Moscow
V. N. Zakutnaya
Research Center of Neurology, Russian Academy of Medical Sciences
Email: shnata@inbox.ru
Russian Federation, Moscow
References
- Гехт Б.М., Ильина Н.А. Нервно-мышечные болезни. М.:Медицина,1982.
- Кузин М.И., Гехт Б.М. Миастения. М.: Медицина, 1996.
- Сепп Е.К., Ланцова В.Б. Миастения. М.: АНО Учебный центр «Невромед-Клиника», 2008.
- Щербакова Н.И., Павлова Е.М., Санадзе А.Г. и др. Клинический случай миастенического криза: возможные механизмы развития, особенности клинического течения и тактика ведения. Неврол. журн. 2010; 3: 35–41.
- Abramsky O., Tarrab-Hazdai R., Aharonov A., Fuchs S. Immunosuppression of experimental autoimmune myasthenia gravis by hydrocortisone and azathioprine. J. Immunol. 1976; 117: 225–228.
- Antonioni G., Bove R., Filippini C., Millefiorini M. Results of open trial of cyclosporine in a group of steroido-dependent myasthenic subjects. J. Clin. Neurol. Nerosurg. 1990; 92: 317–321.
- Aoyama K., Umegae N., Takahashi K. et al. A case of myasthenia gravis with an invasive thymoma. J. Rinsho Shinkeigaku. 1998; 38: 465–467.
- Barohn R.J., McIntire D., Herbelin L. et al. Reliability testing of the quantitative myasthenia gravis score. Ann. N. Y. Acad. Sci. 1998; 841: 769–772.
- Deymeer F., Gungor-Tuncer O., Yilmaz V. et al. Clinical comparison of anti-MuSK- vs anti-AChR-positive and seronegative myasthenia gravis. Neurology 2007; 68: 609–611.
- Frey F.J. Cyclosporine in autoimmune diseases. J. Schweiz Med. Wochenschr. 1990; 120: 772–786.
- Giraud C., Pourrat O., Badia P. et al. Are long-term plasma exchanges and cyclosporin useful in management of severe chronic respiratory failure due to myasthenia gravis? Ann. Intern. Med. 1997; 148: 504–505.
- Goulon M., Elkharrat D., Gajdos P. Treatment of severe myasthenia gravis with cyclosporin. A 12-month open trial. Presse Med. 1989; 18: 341–346.
- Hart I.K., Sharshar T., Sathasivam S. Immunosuppressant drugs for myasthenia gravis. J. Neurol. Neurosurg. Psychiatry 2009; 80: 5–6.
- Kurokawa T., Nishiyama T., Yamamoto R. et al. Anti-MuSK antibody positive myasthenia gravis with HIV infection successfully treated with cyclosporin: a case report. Rinsho Shinkeigaku 2008; 48: 666–669.
- Lavrnic D., Vujic A., Rakocevic-Stojanovic V. et al. Cyclosporine in the treatment of myasthenia gravis. Acta Neurol. Scand. 2005; 111: 247–252.
- Lefvert A.K., Matell G. Antibodies against human cholinergic receptor proteins in patients with myasthenia gravis: studies during immunosupressive treatment. Preliminary report Acta Neurol. Scand. 1977; 201: 181–182.
- Manna R., Verrecchia E., Fonnesu C. et al. Cyclosporine A: good response for patients affected by autoimmune disorders and HCV infection? Eur. Rev. Med. Pharmacol. Sci. 2009; Suppl 1: 63–69.
- Marchiori P.E., De Assis J.L., Scaff M. Failure of treatment of myasthenia gravis by cyclosporin-A: A case report. Arq. Neuropsiquiatr. 1989; 47: 91–93.
- Sanders D.B., Siddiqi Z.A. Lessons from two trials of Mycopfenjlate Mofetil in myasthenia gravis. Ann. N. Y. Acad. Sci. 2008; 1132: 249–253.
- Sanders D.B., Evoli A. Immunosuppressive therapies in myasthenia gravis. Autoimmunity 2010; 43: 1–8.
- Sato A., Katsui T., Honma A. et al. A case of myasthenia gravis treated with cyclosporine and high-dose intravenouse immunoglobulin. J. Nippon Naiaka Gakkai Zasshi. 1996; 85: 279–281.
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