Long-term cyclosporine treatment in a group of severe myasthenia gravis patients

Bonifati, Domenico Marco; Angelini, C.

doi:10.1007/s004150050141

Cited by 57 publications

(38 citation statements)

References 22 publications

(20 reference statements)

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“…It was superior to placebo by 6 months. Cyclosporine A also has been shown to reduce the need for medications or plasmapheresis in several open-label trials in refractory patients [21,22,Class III]. Cost/cost effectiveness Costs run between $300 and $800 per month, depending on the dose used to maintain a response.…”

Section: Cyclosporinementioning

confidence: 98%

Myasthenia gravis

Graves¹,

Katz²

2004

Curr Treat Options Neurol

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Myasthenia gravis is a disorder of neuromuscular transmission, manifest by variable weakness of skeletal muscle. The disorder has a range of therapies that differ with respect to efficacy, timing, and side effects. The physician treating myasthenia gravis must be well versed in understanding the evidence basis for using these agents, as well as the trade-offs between persistent disease manifestations and their costs and expected benefits. Diagnosis of myasthenia gravis depends on recognizing the pattern of weakness, which typically involves some combination of extraocular, bulbar, facial, limb, and neck muscles. Management relies on some combination of medications that influence the function of the neuromuscular junction and treatments that alter the immune response. Thymectomy is commonly used, although trends in evidence-based medicine are leading expert clinicians to look closely at its efficacy. Plasma exchange is useful for patients in crisis, who require rapid improvement. The exact role for high-dose intravenous immunoglobulin in this setting is still being studied, although the agent is gaining popularity. Knowing that the treatments are effective is not enough. It is still important to determine the treatment that has a more rapid onset, because these patients often require intensive care or respiratory assistance.

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Section: Cyclosporinementioning

confidence: 98%

Myasthenia gravis

Graves¹,

Katz²

2004

Curr Treat Options Neurol

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“…Seven out of nine patients (78%) showed marked improvements after a mean of 2 years of treatment. 34 In the third trial, the patients recruited had not responded to thymectomy, steroids and azathioprine. Out of 52 patients, 44 (85%) showed marked improvements after an average follow-up period of 30 months on treatment.…”

Section: Ciclosporin Nonrandomized Evidencementioning

confidence: 99%

Steroids and immunosuppressant drugs in myasthenia gravis

Sathasivam

2008

Nat Rev Neurol

101

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In chronic autoimmune conditions such as myasthenia gravis (MG), immunosuppression--usually long-term--is often necessary. The mechanisms of action of immunosuppressant drugs in MG fall into three main categories: inhibition of the cell cycle (azathioprine, cyclophosphamide, methotrexate and mycophenolate mofetil), immunosuppression of T cells (steroids, ciclosporin and tacrolimus), and B-cell depletion (rituximab). Data on immunosuppressant drugs in MG derive mainly from clinical experience, observational studies and expert opinion. The main drawbacks of the randomized evidence are the small size of most drug trials, variations in study design, and a lack of head-to-head studies. It is therefore difficult to determine the relative efficacy of each immunosuppressant. Oral prednisolone, usually started at a low dose on an alternate-day regimen, and gradually increased, is the recommended first-choice short-term immunosuppressant. Long-term immunosuppression regimens vary between different countries and physicians. Azathioprine is often the first-choice drug for long-term immunosuppression, and it is usually started together with steroids to allow tapering of steroids to the lowest dose possible. Methotrexate, mycophenolate mofetil or tacrolimus should be considered in patients who are intolerant of or unresponsive to azathioprine. Ciclosporin and cyclophosphamide should only be considered as a last resort, as these drugs can cause serious adverse events. Data on rituximab use in MG are sparse, but the initial results are promising.

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“…Myasthenia gravis (MG) is an autoimmune disease that compromises the neuromuscular junction as a result of the activity of anti-acetylcholine receptor antibodies (anti-AchR) on the post-synaptic membrane, leading to symptoms of fatigue and decreased muscle strength [1][2][3][4] . MG is a rare disease with a prevalence of 0.5 to 5 cases per 100,000 habitants and an incidence of 0.4 cases per 100,000.…”

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confidence: 99%

Myasthenic crisis: report of 24 cases

Werneck

Scola

Germiniani

et al. 2002

Arq. Neuro-Psiquiatr.

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-Myasthenic crisis (MC) is a life-threatening complication of myasthenia gravis (MG) with a high mortality rate. The aim of our study was to review the different therapeutics approaches in the treatment of MC and their impact in the final outcome. We reviewed the medical files of patients diagnosed with MG admitted between February 1993 and October 1997, who developed MC. Sex, mean age, disease's duration, functional scale, symptoms preceding the crisis, crisis therapy in each set and mortality were then analysed. There were 24 patients who developed MC, 21 females and 3 males, with 1 neonatal, 1 congenital sporadic, 17 juvenile/adult, 3 over 50 years and 2 with thymoma. Dysphagia, dysphonia and dysartria were the most common symptoms preceding the crisis. A precipitating factor was elicited in 8 cases and the most common was infection (upper airway infection, urinary tract infection and pneumonia). 16 patients needed a nasogastric tube and 9 had a tracheostomy performed. 24 patients used anticholinesterase drugs, 21 prednisone, 7 immunosuppressive agents, 5 plasmapheresis, 3 human hyperimune gamma immunoglobulin and 12 had thymectomy. A good response was obtained in 13, satisfactory in 7 and there were 4 deaths. We concluded that in spite of all the therapeutics options, there were non statistically significant differences in the outcome of patients that underwent thymectomy and those who did not.KEY WORDS: myasthenia gravis, myasthenic crisis, thymectomy, neuromuscular disorders, muscle diseases.Crise miastênica: relato de 24 casos Crise miastênica: relato de 24 casos Crise miastênica: relato de 24 casos Crise miastênica: relato de 24 casos Crise miastênica: relato de 24 casos RESUMO -A crise miastênica (CM) é uma complicação preocupante da miastenia grave (MG) que apresenta altos índices de mortalidade. Neste estudo revisamos as diferentes abordagens no tratamento da CM e seu impacto no resultado final. Levantamos os dados dos pacientes com MG que desenvolveram CM admitidos entre fevereiro de 1993 e outubro de 1997. Foram analisados as interrelações do sexo, idade media, duração da doença, escala funcional, sintomas e procedimentos precedendo as crises, terapêutica empregada e mortalidade. Foram encontrados 24 casos que desenvolveram CM, sendo 21 do sexo feminino e 3 do masculino. Em relação à apresentação clínica, 1 era da forma neonatal, 1 congênita esporádica, 17 juvenil/adulta, 3 acima de 50 anos e 2 com timoma. Os sintomas principais que precederam a CM foram disfagia, disfonia e disartria. Foi possível identificar um fator desencadeante em 8 casos e o mais comum foi infecção (vias aéreas, pneumonia e trato urinário). Necessitaram de sonda nasogastrica 16 casos e a traqueostomia, 9. Usaram medicações anticolinesterásicas todos os 24 casos, prednisona 21, imunossupressivos 7, plasmaferese 5, gamaglobulina hiperimune 3 e foram submetidos a timectomia 12 casos antes ou após a CM. Obtivemos bom resultado em 13, satisfatório em 7 e ocorreram 4 óbitos, sendo 3 não relacionados com a CM. Concluímos que...

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Long-term cyclosporine treatment in a group of severe myasthenia gravis patients

Cited by 57 publications

References 22 publications

Myasthenia gravis

Myasthenia gravis

Steroids and immunosuppressant drugs in myasthenia gravis

Myasthenic crisis: report of 24 cases

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