Long-term treatment of refractory myasthenia gravis with subcutaneous immunoglobulin

Kovács, Eszter; Dankó, Katalin; Nagy-Vince, Melinda; Csiba, László; Boczán, Judit

doi:10.1177/1756285617722437

Cited by 10 publications

(5 citation statements)

References 12 publications

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“…Steroids are associated with several well-documented serious long-term AEs (Table S2), including osteoporosis and diabetes. 49,53 Treatment strategies in patients with MG aim to reduce the steroid dose when possible through the use of steroid-sparing immunosuppressants (azathioprine, MMF, ciclosporin, tacrolimus, and methotrexate). However, these are themselves associated with significant AEs.…”

Section: Burden Of Refractory Myasthenia Gravismentioning

confidence: 99%

Understanding the burden of refractory myasthenia gravis

Schneider‐Gold

Hagenacker

Melzer

et al. 2019

Ther Adv Neurol Disord

107

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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: Burden Of Refractory Myasthenia Gravismentioning

confidence: 99%

Understanding the burden of refractory myasthenia gravis

Schneider‐Gold

Hagenacker

Melzer

et al. 2019

Ther Adv Neurol Disord

107

View full text Add to dashboard Cite

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“…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%

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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“…To date, this has not been formally evaluated in MG for SCIg per se . A single reported case achieved stabilization of MG with 0.16 g/kg per week ( 4 ). In looking beyond SCIg in MG, there is no evidence of a difference in efficacy between 1 and 2 g/kg dosing regimens for IVIg in MG exacerbation ( 17 ).…”

Section: Discussionmentioning

confidence: 99%

“…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 – 8 ).…”

Section: Introductionmentioning

confidence: 99%

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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Long-term treatment of refractory myasthenia gravis with subcutaneous immunoglobulin

Cited by 10 publications

References 12 publications

Understanding the burden of refractory myasthenia gravis

Understanding the burden of refractory myasthenia gravis

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

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

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