Extracorporeal Immunoglobulin Elimination for the Treatment of Severe Myasthenia Gravis

Bláha, M.; Piťha, Jiří; Bláha, V.; Lánska, M.; Malý, J; Filip, Stanislav; Langrová, H

doi:10.1155/2010/419520

Cited by 6 publications

(2 citation statements)

References 18 publications

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“…26 In addition, a prospective observational study in patients with severe refractory MG [Myasthenia Gravis Foundation of America (MGFA) classification 27 IIIb-IVb; n = 6] reported two to five myasthenic crises requiring artificial ventilation in each of two patients, and more than five such crises in each of two patients; the remaining two patients had one or two crises. 28 In the phase III study of eculizumab in patients with refractory generalized MG, 78% of patients had a history of MG exacerbations and 18% had experienced a myasthenic crisis in the 2 years before study initiation. Furthermore, almost a quarter of patients with refractory generalized MG had previously required ventilator support during the course of their MG. 29 The experiences reported in these studies are supported by an analysis of health plan databases conducted in the United States of America (USA; refractory MG, n = 403; nonrefractory MG, n = 3811; non-MG control patients, n = 403).…”

Section: Number Definition Attributesmentioning

confidence: 99%

Understanding the burden of refractory myasthenia gravis

Schneider‐Gold

Hagenacker

Melzer

et al. 2019

Ther Adv Neurol Disord

106

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Myasthenia gravis (MG) is an autoantibody-mediated disease that compromises the acetylcholine receptors or associated structures of the postsynaptic membrane of the neuromuscular junction. This leads to impaired neuromuscular transmission and subsequent fluctuating fatigability and weakness of ocular, bulbar, and limb skeletal muscles. Over the past few decades, there have been significant advances in our understanding of the disease pathophysiology and improvements in prognosis due to intensive care medicine and immunomodulation. Despite this, an estimated 10–20% of patients with MG do not achieve an adequate response, are intolerant to conventional treatment, or require chronic treatment with intravenous immunoglobulins or plasma separation procedures. Such patients are regarded as having MG that is ‘refractory’ to treatment and may represent a distinct clinical subgroup. Because the majority of patients with MG have well-controlled disease, the burden of illness in the minority with refractory disease is poorly understood and may be underestimated. However, clinically these patients are liable to experience extreme fatigue, considerable disability owing to uncontrolled symptoms, and frequent myasthenic crises and hospitalizations. Both acute adverse effects and an increased risk of comorbidity from treatment regimens may contribute to reduced quality of life. As yet, little is known concerning the impact of refractory MG on mental health and health-related quality of life. This review aims to highlight the burden of disease and unmet needs in patients with refractory MG.

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Section: Number Definition Attributesmentioning

confidence: 99%

Understanding the burden of refractory myasthenia gravis

Schneider‐Gold

Hagenacker

Melzer

et al. 2019

Ther Adv Neurol Disord

106

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“…Standard plasma exchange has several disadvantages (it is nonselective and there is a danger of allergic reactions and infection transmission after volume substitution with fresh frozen plasma) and is currently replaced by more selective and safer methods, for example, plasma filtration15 or immunoapheresis 13. We decided to use the plasma filtration technique because it has been proven to be effective in other autoimmune disorders, such as myasthenia gravis,17, 22 Guillain‐Barre's, and Fisher's syndromes,16 and for removal of ABO antibodies before renal transplantation 23…”

Section: Discussionmentioning

confidence: 99%

Plasma filtration in the treatment of graves' ophthalmopathy: A randomized study

Ceeová

Skacha

et al. 2010

J of Clinical Apheresis

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The aim of this study was to perform a randomized study to evaluate the role of plasma filtration in the treatment of severe thyroid-associated ophthalmopathy (TAO). 20 patients were enrolled, and all patients were treated with methylprednisolone IV pulses. 10 randomly chosen patients were also subjected to plasma filtration (twice weekly in Weeks 1, 2, 4, 7, and 10). The procedure proved to be safe. All immunoglobulin classes as well as autoantibodies directed against thyroglobulin, thyroid peroxidase, and TSH receptor exhibited statistically significantly decreases. Some markers of cell-mediated immunity such as soluble antigen CD30 and monocyte chemotactic protein 1 decreased, but serum levels of other markers such as CD40 ligand and soluble protein Fas/Apo-1 did not change significantly. The decrease of immunoglobulins was long lasting, whereas cytokine levels returned to basal values before the next apheresis. Although the clinical activity score (CAS) dropped in all patients, it occurred more rapidly in patients treated with plasma filtration. The CAS difference between the two groups was statistically significant (p = 0.027). The amplitude of visual evoked potentials improved after 3 months in the plasma filtration group. At the end of the study, there was no difference between patients treated with aphereses and the control group. Eye muscle width and proptosis measured by CT scan did not differ between the two groups. We conclude that apheresis can decrease disease activity more rapidly than standard high-dose IV glucocorticoid therapy. Whether this superior treatment effect could potentially avoid surgical procedures remains to be determined.

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