Immunomodulatory effects and clinical benefits of intravenous immunoglobulin in myasthenia gravis

Figures & data

Figure 1. Main factors involved in the immunopathogenesis of myasthenia gravis that are relevant to the actions of intravenous immunoglobulin. Reproduced with permission from [2]. IVIg has effects on antibodies (1a,b,c,d), complement (2), cytokines (3a,b), FcγRIIB (4), and T-cell and APC functions (5a,b). It affects antibodies by providing idiotypic antibodies, facilitating neutralization of pathogenic autoantibodies (1a), by affecting related cytokines (1b), by suppressing B-cell trophic factors, such as B-cell activating factor (BAFF) (1c), and by accelerating the catabolism of pathogenic immunoglobulin G (IgG) by saturating the FcRn transport receptors (1d). IVIg also: inhibits complement binding (2) and prevents membranolytic attack complex (MAC) formation; suppresses pathogenic cytokines (3a,b); upregulates FcγRIIb inhibitory receptors (4), intercepting antibody-dependent cell-mediated cytotoxicity; has effects on antigen presenting cells, T-cell modulatory functions and antigen recognition (5a,b); and affects immunoregulatory genes. AChR, acetylcholine receptor; APC, antigen-presenting cell; BAFF, B-cell activating factor; IL, interleukin; MHC, major histocompatibility complex; TCR, T-cell receptor; Treg, regulatory T cell.

Figure 2. Efficacy of IVIg-C in patients with exacerbation of myasthenia gravis: change in Quantitative Myasthenia Gravis (QMG) score from baseline to day 14. Data from [22]. IVIg-C = immune globulin caprylate/chromatography purified.

Figure 3. Efficacy of IVIg-C in patients with exacerbation of myasthenia gravis: percentage of participants with a clinical improvement from baseline to day 14 (QMG decrease ≥3 points; MG Composite decrease ≥3 points; MG ADL decrease ≥2 points). Data from [22]. IVIg-C = immune globulin caprylate/chromatography purified; MG-ADL = Myasthenia Gravis Activities of Daily Living profile; MG Composite = Myasthenia Gravis composite; QMG = Quantitative Myasthenia Gravis score.

Dalakas MC. Progress in the therapy of myasthenia gravis: getting closer to effective targeted immunotherapies. Curr Opin Neurol. 2020;33(5):545–552.

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Karelis G, Balasa R, De Bleecker JL, et al., A phase 3 multicenter, prospective, open-label efficacy and safety study of immune globulin (human) 10% caprylate/chromatography purified in patients with myasthenia gravis exacerbations. Eur Neurol. 2019; 81(5–6): 223–230.

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