Subcutaneous Immunoglobulins are a Valuable Treatment Option in Myasthenia Gravis

Garnero, Martina; Fabbri, Sabrina; Gemelli, Chiara; Benedetti, Luana; Mancardi, Gianluigi; Schenone, Angelo; Grandis, Marina

doi:10.3988/jcn.2018.14.1.98

Cited by 6 publications

(6 citation statements)

References 4 publications

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“…We previously reported the results of an open-label prospective trial of SCIg 2 g/kg total in mild to moderate exacerbations of MG that was conducted over 6 weeks with assessments at baseline, weeks 2, 4, and 6 (end of study) wherein we demonstrated that SCIg is effective, safe, and tolerable (3). A case report demonstrated stabilization and maintenance with SCIg alone in one of two patients with MG (4), while a growing body of evidence supports the efficacy of SCIg in maintenance of stable MG (2,(5)(6)(7)(8).…”

Section: Introductionmentioning

confidence: 69%

“…Considering that we demonstrated early and continued clinically meaningful improvement in all the disease scores evaluated, there is rationale for a strategy that employs sustained dosing in order to provide patients an adequate therapeutic trial before discontinuing SCIg. While our trial did not include long-term follow-up data, others have shown SCIg is a viable maintenance therapy in MG (2,(5)(6)(7)(8). Adding to this, it is clear that SCIg is safe and tolerable, which lends further support to providing an adequate therapeutic trial for a given patient.…”

Section: Discussionmentioning

confidence: 88%

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Pharmacodynamic Properties of Subcutaneous Immunoglobulin in Myasthenia Gravis: Sub-analyses From an Open-Label Trial

2020

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Background: We previously reported an open-label prospective trial of subcutaneous immunoglobulin (SCIg) in mild to moderate exacerbations of myasthenia gravis (MG). The effective dose of SCIg in MG and whether measured immunoglobulin G (IgG) levels correlated with measures of disease burden were not reported. Objectives: To understand the relationship between SCIg dosing and serum IgG levels on measures of disease burden: quantitative MG (QMG), MG activities of daily living (MG-ADL), MG composite (MGC), and manual muscle testing (MMT) scores. Methods: We performed post-hoc analyses of variance to assess change in oculobulbar and generalized sub-scores. We assessed the improvement in QMG, MG-ADL, MGC, or MMT over intervals from baseline to week 2, weeks 2-4, and week 4 to end of study. Improvement was either greater than (coded 1) or was equal to or less than (coded 0) the previous 2 weeks. Binaries were assessed in binary logistic regression as a function of SCIg dose over the two-week interval as the independent variable. We also performed linear regression analyses with change in the clinical scores as the dependent variable and change in IgG level over the entire study period and over the interval from weeks 2 to 4, during which change in IgG level was maximal, as the independent variables. Results: Subanalysis of QMG and MG-ADL scores demonstrated significant reductions in the oculobulbar and the generalized portions of both measures. Binary logistic regression analyses did not find any statistically significant correlations between the odds of improvement and weight-adjusted dose of SCIg over 2-week intervals. There were no significant relationships between changes in scores and IgG level over the entire study period or over the interval from weeks 2 to 4. Conclusions: Although SCIg dose varied over the study period, the odds of improvement were not significantly correlated with this, which suggests that the current dose of 2 g/kg for SCIg should be compared to different, possibly lower, dosing regimens head-to-head. The change in clinical scores was not significantly associated with IgG levels suggesting a complex relationship. SCIg may be effective for both ocular and generalized presentations of MG.

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Section: Introductionmentioning

confidence: 69%

Section: Discussionmentioning

confidence: 88%

Pharmacodynamic Properties of Subcutaneous Immunoglobulin in Myasthenia Gravis: Sub-analyses From an Open-Label Trial

2020

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“…In our study, the mean duration of evolution was 76.5 months (6.4 years), which was comparable to the study by Beecher et al (77 months) [ 5 ]. In contrast, the mean duration of evolution was much higher in the studies of Alcantara et al (14.5 years) [ 9 ], Bourque et al (11.8 years) [ 8 ] and in the case reported by Garnero et al (22 years) [ 10 ].…”

Section: Discussionmentioning

confidence: 86%

“…Nevertheless, our cohort was of similar or even larger size than those published. Specifically, two studies included between 20 and 30 patients [5,9], one study included less than 10 patients [8], and three publications were case reports [10][11][12]. In addition, our sample included patients with various profiles in terms of age, duration of disease progression, type of antibodies, and number of immunosuppressants tried.…”

Section: Discussionmentioning

confidence: 99%

“…However, the data focusing on SCIg in MG are limited. We identified only 2 prospective open-label trial (23 patients) [5][6][7], 2 retrospective studies (9 patients [8]-34 patients [9]) and 3 published case reports [10][11][12]. Although SCIg treatment seems to be an interesting alternative treatment to IVIg and a complementary treatment to immunosuppressants, there is no official recommendation for its use as a chronic maintenance therapy in MG due to a lack of data and studies.…”

Section: Introductionmentioning

confidence: 99%

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Usefulness of subcutaneous immunoglobulin therapy in the management of myasthenia gravis: a retrospective cohort study

et al. 2022

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Introduction To describe the efficacy of subcutaneous immunoglobulin (SCIg) in patients with myasthenia gravis (MG). Methods This was a retrospective study conducted in the neuromuscular referral center of Bordeaux (between January 1, 2014 and March 31, 2021) with MG patients treated with SCIg. The main outcome was SCIg efficacy assessed by the before and after SCIg Myasthenia Gravis Foundation of America (MGFA) clinical classification, the duration of hospitalization and the number of days of orotracheal intubation (OTI). Results Sixteen patients were included in the study (11 females; 5 males). Nine patients were still treated with SCIg at the end of the study (March 31, 2021) and then underwent prospective follow-up. The average age of the patients was 56.1 (19–83) years. The median duration of MG at onset of SCIg was 37.4 months. Eight patients (50%) remained stable (4 in stage MGFA-IV and 4 in MGFA-III). Eight patients (50%) improved: 3 from MGFA-IV to MGFA-III, 1 from MGFA-IV to MGFA-II, 1 from MGFA-IV to MGFA-I, 2 from MGFA-III to MGFA-II and 1 from MGFA-III to MGFA-I (no patient worsened). The duration of disease progression did not appear to affect the response to SCIg therapy. The number of hospital days per month was significantly reduced after SCIg compared to before, and the number of days in intensive care unit and the number of days of OTI were also reduced. Only minor adverse effects were noted, and 80% of patients were in favor of continuing SCIg. Conclusions SCIg is a well-tolerated and useful treatment in MG, offering interesting perspectives in the management of MG patients. However, further large-scale prospective studies are needed to confirm these results.

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Efficacy and tolerability of subcutaneously administered immunoglobulin in myasthenia gravis: A systematic review

Adiao

Espiritu

Roque

et al. 2020

Journal of Clinical Neuroscience

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Subcutaneous Immunoglobulins are a Valuable Treatment Option in Myasthenia Gravis

Cited by 6 publications

References 4 publications

Pharmacodynamic Properties of Subcutaneous Immunoglobulin in Myasthenia Gravis: Sub-analyses From an Open-Label Trial

Pharmacodynamic Properties of Subcutaneous Immunoglobulin in Myasthenia Gravis: Sub-analyses From an Open-Label Trial

Usefulness of subcutaneous immunoglobulin therapy in the management of myasthenia gravis: a retrospective cohort study

Efficacy and tolerability of subcutaneously administered immunoglobulin in myasthenia gravis: A systematic review

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