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

Adiao, Karen Joy; Espiritu, Adrian I.; Roque, Vladimir Lennin A.; Reyes, Jose Paciano

doi:10.1016/j.jocn.2019.08.113

Cited by 7 publications

(8 citation statements)

References 25 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: 66%

“…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%

“…Furthermore, a phase 3 trial in maintenance treatment of chronic inflammatory demyelinating polyradiculoneuropathy with SCIg that compared high dose (0.4 g/kg per week), low dose (0.2 g/kg per week), and placebo found significantly less relapses in the treatments groups compared to placebo, but no significant difference between treatment groups (18). Beyond the obvious benefit of lower doses conferring a lower likelihood of side effects, there remains doubt about the role of SCIg in severe exacerbations and crises given the infusion volumes and infusion times required (5), which could potentially be ameliorated by lower dosing requirements.…”

Section: Discussionmentioning

confidence: 99%

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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: 66%

Section: Discussionmentioning

confidence: 88%

Section: Discussionmentioning

confidence: 99%

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

2020

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“…Patients requiring chronic IVIG can frequently be transitioned to SCIG and this therapy is gaining popularity in autoimmune neuromuscular disorders. A recent systematic review on the efficacy and safety of SCIG in chronic MG identified five studies that showed improvement across several MG measures including MG QOL, MG ADL, stability of MRC sum score and reduction in PLEX requirement [ 116 ]. A recent retrospective study showed significant reduction in the MG impairment index (MGII) in patients on SCIG, as well as reduced doses of corticosteroid and AChEI [ 117 ].…”

Section: Current Immunosuppressive Treatmentmentioning

confidence: 99%

Pharmacotherapy of Generalized Myasthenia Gravis with Special Emphasis on Newer Biologicals

Menon

Bril

2022

Drugs

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Myasthenia gravis (MG) is a chronic, fluctuating, antibody-mediated autoimmune disorder directed against the post-synaptic neuromuscular junctions of skeletal muscles, resulting in a wide spectrum of manifestations ranging from mild to potentially fatal. Given its unique natural course, designing an ideal trial design for MG has been wrought with difficulties and evidence in favour of several of the conventional agents is weak as per current standards. Despite this, acetylcholinesterases and corticosteroids have remained the cornerstones of treatment for several decades with intravenous immunoglobulins (IVIG) and therapeutic plasma exchange (PLEX) offering rapid treatment response, especially in crises. However, the treatment of MG entails long-term immunosuppression and conventional agents are viable options but take longer to act and have a number of class-specific adverse effects. Advances in immunology, translational medicine and drug development have seen the emergence of several newer biological agents which offer selective, target-specific immunotherapy with fewer side effects and rapid onset of action. Eculizumab is one of the newer agents that belong to the class of complement inhibitors and has been approved for the treatment of refractory general MG. Zilucoplan and ravulizumab are other agents in this group in clinical trials. Neisseria meningitis is a concern with all complement inhibitors, mandating vaccination. Neonatal Fc receptor (FcRn) inhibitors prevent immunoglobulin recycling and cause rapid reduction in antibody levels. Efgartigimod is an FcRn inhibitor recently approved for MG treatment, and rozanolixizumab, nipocalimab and batoclimab are other agents in clinical trial development. Although lacking high quality evidence from randomized clinical trials, clinical experience with the use of anti-CD20 rituximab has led to its use in refractory MG. Among novel targets, interleukin 6 (IL6) inhibitors such as satralizumab are promising and currently undergoing evaluation. Cutting-edge therapies include genetically modifying T cells to recognise chimeric antigen receptors (CAR) and chimeric autoantibody receptors (CAAR). These may offer sustained and long-term remissions, but are still in very early stages of evaluation. Hematopoietic stem cell transplantation (HSCT) allows immune resetting and offers sustained remission, but the induction regimens often involve serious systemic toxicity. While MG treatment is moving beyond conventional agents towards target-specific biologicals, lack of knowledge as to the initiation, maintenance, switching, tapering and long-term safety profile necessitates further research. These concerns and the high financial burden of novel agents may hamper widespread clinical use in the near future.

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“…The available randomized controlled trials and long‐term open‐label data suggest that erenumab, a fully human monoclonal antibody directed against the CGRPr, is a safe and effective treatment for migraine . SCIg can be used in patients with severe or rapidly worsening MG and act as immunomodulators by neutralizing circulating autoantibodies . Hypothetically, SCIg may also inactivate therapeutic monoclonal antibodies.…”

mentioning

confidence: 99%

Erenumab Plus Subcutaneous Immunoglobulin in a Patient With Comorbid Chronic Migraine and Myasthenia Gravis

et al. 2020

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

Cited by 7 publications

References 25 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

Pharmacotherapy of Generalized Myasthenia Gravis with Special Emphasis on Newer Biologicals

Erenumab Plus Subcutaneous Immunoglobulin in a Patient With Comorbid Chronic Migraine and Myasthenia Gravis

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