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
- 作者: 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
-
隶属关系:
- Research Center of Neurology, Russian Academy of Medical Sciences
- The A.I. Burnazian Federal Medical Biophysical Center, Federal Medical Biological Agency
- 期: 卷 7, 编号 2 (2013)
- 页面: 4-10
- 栏目: Original articles
- URL: https://ogarev-online.ru/2075-5473/article/view/124560
- DOI: https://doi.org/10.17816/psaic242
- ID: 124560
如何引用文章
全文:
作者简介
N. Shcherbakova
Research Center of Neurology, Russian Academy of Medical Sciences
编辑信件的主要联系方式.
Email: shnata@inbox.ru
俄罗斯联邦, Moscow
E. Pavlova
The A.I. Burnazian Federal Medical Biophysical Center, Federal Medical Biological Agency
Email: shnata@inbox.ru
俄罗斯联邦, Moscow
V. Lantsova
Research Center of Neurology, Russian Academy of Medical Sciences
Email: shnata@inbox.ru
俄罗斯联邦, Moscow
N. Savitskaya
Research Center of Neurology, Russian Academy of Medical Sciences
Email: shnata@inbox.ru
俄罗斯联邦, Moscow
E. Pavlov
Research Center of Neurology, Russian Academy of Medical Sciences
Email: shnata@inbox.ru
俄罗斯联邦, Moscow
I. Retinskaya
Research Center of Neurology, Russian Academy of Medical Sciences
Email: shnata@inbox.ru
俄罗斯联邦, Moscow
V. Zakutnaya
Research Center of Neurology, Russian Academy of Medical Sciences
Email: shnata@inbox.ru
俄罗斯联邦, Moscow
参考
- 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.
补充文件
