Plasmapheresis in Refractory Generalized Myasthenia Gravis

Kornfeld, Peter; Ambinder, Edward P.; Mittag, Thomas W.; Bender, Adam N.; Papatestas, Angelos E.; Goldberg, Judith; Genkins, Gabriel

doi:10.1001/archneur.1981.00510080040003

Cited by 63 publications

(29 citation statements)

References 12 publications

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“…The immediate improvement of clinical symptoms in Myasthenia gravis reflects the dynamic redistribution of AChRAb between neuromuscular synapses and intravascular spaces [23,24]; however, unlike in other diseases which directly depend on the absolute concentration of pathogenic factors, in Myasthenia gravis the absolute serum level of anti AChRAb has no strict correlation with disease severity especially in acute situations. Using an additional combined immunosuppressive strategy with corticosteroids and azathioprine, stable clinical improvement initiated by IA and PE was achieved despite AChRAb rebound of plasma levels 1 to 3 weeks after the last removal suggesting no direct correlation of MG severity and Ab concentration [22,31,33,35,36]. Potentially, not only the removal of anti-AChR antibodies but also of other pathogenic factors, i.e., cellular effects [24,27], antibody species undetectable with current test systems [2,8,11] and the removal of immune complexes with complement, might contribute to the treatment effect in myasthenic crisis using broad immunoglobulin elimination techniques like plasma exchange or tryptophan mediated immunoadsorption.…”

Section: Discussionmentioning

confidence: 99%

A randomized and controlled study comparing immunoadsorption and plasma exchange in myasthenic crisis

Köhler

Bucka

Klingel

2011

J of Clinical Apheresis

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Myasthenic crisis is the most serious life-threatening event in Myasthenia gravis patients, affecting up to 27% within the first two years after onset of disease. Extracorporeal removal of circulating autoantibodies against the nicotinic acetylcholine receptor (AChRAb) by methods of therapeutic apheresis, e.g. plasma exchange (PE) and immunoadsorption (IA) had been demonstrated as effective treatment especially in acute situations of myasthenic crisis. However, controlled data comparing clinical safety and efficacy of both methods in a clinical study were not available. Here the results of a prospective randomized controlled clinical trial are presented, investigating 19 patients with myasthenic crisis, who were randomized to receive either PE (n = 10) or IA (n = 9) in addition to combined drug treatment. Patients received 3 to 5 (mean 3.5 for PE, and 3.4 for IA) treatments over a period of 7 days with a predefined treatment volume of 1.5 l plasma (i.e., 20-25 ml/kg plasma representing 0.5-0.6 patients' plasma volumes). Clinical courses were monitored using disease specific clinical scores. After initiation of IA or PE the mean value of Myasthenia scores decreased equally until Day 14 of the post-treatment phase. Patients from both treatment groups improved to a stable clinical status of Oosterhuis Classes 1 and 2. Substantial reduction of AChRAb was documented after each session of PE or IA. In the treatment period 16 adverse effects (seven serious adverse events, SAE) in the PE and 10 (1 SAE) in the IA group were observed. In conclusion, IA proved to be equally effective compared with PE treatment in patients with myasthenic crisis. Three to five treatment sessions using low plasma volume dosage of 20-25 ml/kg were adequate to improve clinically relevant symptoms significantly in most patients.

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Section: Discussionmentioning

confidence: 99%

A randomized and controlled study comparing immunoadsorption and plasma exchange in myasthenic crisis

Köhler

Bucka

Klingel

2011

J of Clinical Apheresis

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“…They had 6 and 5 cycles of PE respectively, in a monthly schedule of two consecutive days sessions. Chronic PE was proposed by Kornfeld et al 6 as effective therapy in cases of generalized MG refractory to other treatments. Rodnitzky and Bosch 5 described two cases of intermittent PE therapy prescribed up to five years without significant side effects.…”

Section: Resultsmentioning

confidence: 99%

“…The two commonest indications for acute PE are myasthenic crisis and in the preparation for thymectomy 3 . Chronic PE is a term to be used when the procedure is performed on a regular basis 4 for a long interval 5 , and it is proposed for general-ized MG refractory to other treatments 6 . PE is a therapeutic possibility in seropositive and seronegative forms of MG 7 .…”

Section: Resumo -Analisamos a Experiência Do Hospital Das Clínicas Damentioning

confidence: 99%

Plasmapheresis in the treatment of myasthenia gravis: retrospective study of 26 patients

Carandina-Maffeis

Nucci

Marques

et al. 2004

Arq. Neuro-Psiquiatr.

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-We analyzed the experience of Unicamp Clinical Hospital with plasma exchange (PE) therapy in myasthenia gravis (MG). About 17.8 % of a totality of MG patients had PE performed: 26 cases, 19 women and seven men. The mean age-onset of MG was 28 years, extremes 11 and 69. Minimum deficit observed in the group was graded IIb (O & G) or IIIa (MGFA scale). One patient had prethymectomy PE. In seven the procedures were performed due to myasthenic crisis and in 18 patients due to severe myasthenic symptoms or exacerbation of previous motor deficit. Two patients were also submitted to chronic PE considering refractoriness to other treatments. Twenty-six patients had 44 cycles of PE and 171 sessions. The mean number of sessions was 3.9 (SD ± 1.4) each cycle; median 5, extremes 2 and 6. The mean time by session was 106,5 minutes (SD ± 35.2); median 100.5 (extremes of 55 and 215).The mean volume of plasma exchanged in each session was 2396 ml (SD ± 561); median 2225 (extremes 1512 and 4500). Side effects occurred: reversible hypotension (seven cases), mild tremor or paresthesias (seven cases). Infection and mortality rates due to PE were zero. All patients had immediate benefit of each PE cycle and usually they also received prednisone or other immunosuppressors. Good acceptance of the procedure was observed in 80.7% of patients.KEY WORDS: plasmapheresis, myasthenia gravis, therapeutics, acceptance, complications. Plasmaférese no tratamento da miastenia grave: estudo retrospectivo de 26 pacientes RESUMO -Analisamos a experiência do Hospital das Clínicas da Unicamp com plasmaferese: (PF) na miastania grave (MG). 17,8 % do total dos casos de MG submeteu-se a PF, 26 casos, 19 mulheres e sete homens. A idade média de início da MG foi 28 anos (extremos 11 e 69). O menor déficit clínico foi IIb (O & G) e IIIa (MGFA).A PF foi indicada no pré-operatório de timectomia em um caso e em sete devido a crise miastênica. Em 18 casos, com MG generalizada e sintomas bulbares ou com exacerbação de déficit prévio, a PF foi indicada como intervenção aguda. Em dois pacientes desse grupo ela foi indicada também em regime crônico de ciclos mensais. Os 26 pacientes submeteram-se a 44 ciclos e a 171 sessões de PF. O número médio de sessões em cada ciclo foi 3,9 (DP ± 1,4); mediana de 5, extremos 2 e 6. O tempo médio de cada sessão foi 106,5 minutos (DP ± 35,2); mediana de 100,5 (extremos 55 e 215).O volume médio de plasma trocado em cada sessão foi 2396 ml (DP ± 561); mediana 2225 (extremos 1512 e 4500). Efeitos colaterais foram reversíveis: hipotensão (sete casos), tremor ou parestesias leves (sete casos). Taxas de infecção e mortalidade devido a PF foram zero. A totalidade dos pacientes teve benefícios imediatos a cada ciclo de PF e usualmente receberam prednisona ou outro imunossupressor. Houve boa aceitação ao procedimento em 80,7% dos pacientes.

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“…Sinai showed excellent clinical responses in 12 patients [7]. That clinical study by Kornfeld is an excellent model study with extensive clinical data and semi-quantitative evaluations, requiring at least one clinical grade category of improvement to note an effect where nine of nine in a subset improved.…”

Section: Discussionmentioning

confidence: 98%

“…It soon became obvious that Myasthenia Gravis (MG), which needed more than standard therapies, would provide the niche for PEX [6]. When MG is not controlled with anti-cholinesterase, thymectomy, and immunosuppressive therapy, PEX will facilitate management of the disease [7][8][9][10] and markedly shorten the duration of a crisis.…”

Section: Introductionmentioning

confidence: 99%

Predictable recovery from myasthenia gravis crisis with plasma exchange: Thirty-six cases and review of current management

et al. 1999

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Plasmapheresis in Refractory Generalized Myasthenia Gravis

Cited by 63 publications

References 12 publications

A randomized and controlled study comparing immunoadsorption and plasma exchange in myasthenic crisis

A randomized and controlled study comparing immunoadsorption and plasma exchange in myasthenic crisis

Plasmapheresis in the treatment of myasthenia gravis: retrospective study of 26 patients

Predictable recovery from myasthenia gravis crisis with plasma exchange: Thirty-six cases and review of current management

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