Abstract
Introduction
Myasthenia gravis (MG) is an autoimmune neurological disorder affecting the neuromuscular junction. Rapid clinical deterioration during a myasthenic crisis (MC) or severe exacerbation may be life-threatening due to respiratory or bulbar involvement. Plasma exchange (PLEX) and intravenous immunoglobulin (IVIg) are two rescue therapies that show comparable efficacy in this setting. However, the characteristics of patients who respond poorly to IVIg remain insufficiently described. The aim of this study was to identify predictive factors of non-response to IVIg administered during a first MC or exacerbation.
Methods
This was a single-centre retrospective cohort study carried out at a French referral centre for neuromuscular diseases between 2017 and 2023. Adult patients with generalised MG experiencing a first MC or exacerbation and treated for the first time with IVIg were included in the study. Data on clinical, laboratory, electrophysiological and therapeutic variables were collected. Treatment response was defined as the maximum gain in Garches clinical score following IVIg, adjusted for baseline score.
Results
A total of 93 were included in the study. The median age of the cohort was 68 (interquartile range [IQR] 49–76) years, and the median delay from diagnosis was 0.7 years. The median baseline Garches score was 66/100 (IQR 55–75) points, which improved to 85/100 (IQR 75–94) points after treatment. Bulbar involvement was significantly associated with a greater response to IVIg.
Conclusions
In this large and heterogeneous cohort, IVIg demonstrated consistent efficacy without identification of significant predictors of non-response. These findings support IVIg as a reliable and evidence-based first-line therapy for patients with MG experiencing exacerbation or MC.
Supplementary Information
The online version contains supplementary material available at 10.1007/s40120-026-00941-z.
Keywords: Myasthenia gravis, Polyvalent intravenous immunoglobulins, Myasthenic crisis, Myasthenic exacerbation, Garches clinical score, Predictive factors
Key Summary Points
| Why carry out this study? |
| Myasthenic crisis and severe exacerbations in generalised myasthenia gravis (MG) require rapid treatment to prevent respiratory failure and life-threatening complications. Intravenous immunoglobulin (IVIg) and plasma exchange are both recommended rescue therapies |
| Predictive factors of poor response to IVIg remain poorly characterised, which is particularly relevant in the context of emerging targeted therapies, such as complement inhibitors and FcRN antagonists, and global IVIg shortages |
| The aim of this study was to identify clinical, biological, electrophysiological and baseline patient characteristics associated with non-response to first-line IVIg in patients with generalised MG experiencing a first myasthenic crisis or exacerbation, as identifying predictors of non-response could support the selection of alternative first-line treatments |
| What was learned from the study? |
| In a cohort of 93 patients, IVIg led to clinical improvement in 87.1% of cases, with a median gain of 15 points on the Garches clinical score. Bulbar involvement was associated with a significantly greater response to treatment. No independent predictors of non-response were identified |
| These findings confirm IVIg as a reliable and broadly effective first-line therapy for myasthenic exacerbation or crisis across heterogeneous presentations, in the context of emerging therapeutic alternatives such as complement inhibitors and FcRn antagonists |
Introduction
Myasthenia gravis (MG) is an autoimmune disorder of the neuromuscular junction targeting postsynaptic components, leading to impaired neuromuscular transmission [1]. The disease is characterised by fluctuating skeletal muscle weakness affecting ocular, bulbar, axial and limb muscles [2, 3]. Clinical manifestations significantly impair daily functioning and may become life-threatening during exacerbations or a myasthenic crisis (MC), often precipitated by triggering factors [3, 4]. MCs occur most frequently within the first 3 years following diagnosis and are defined by rapid clinical deterioration over 2 to 5 days, potentially progressing to respiratory failure or severe bulbar involvement requiring intensive care management. In approximately 60% of MC cases, the patient requires admission to the intensive care unit (ICU) [4]. In this context, rescue therapies, including plasma exchange (PLEX) and intravenous immunoglobulin (IVIg), provide rapid clinical improvement [5]. Both treatments have demonstrated comparable efficacy and are widely used in current practice [6]. IVIg exerts multifactorial mechanisms, including neutralisation of pathogenic autoantibodies, inhibition of complement activation, downregulation of proinflammatory cytokines and chemokines, modulation of dendritic cell function and saturation of neonatal Fc receptors leading to accelerated degradation of pathogenic immunoglobulin G (IgG) [7, 8]. Unlike PLEX, IVIg can be administered via peripheral venous access in conventional care settings, facilitating rapid use in routine clinical practice. IVIg is also administered during severe exacerbations in patients insufficiently controlled with background therapy and in those with contraindications to corticosteroids, or early in the disease course to achieve rapid improvement while awaiting the delayed effects of immunosuppressive therapy. In cases of IVIg failure, rescue PLEX may be required [9].
With the emergence of novel therapeutic agents such as neonatal Fc receptor (FcRn) inhibitors and complement inhibitors, [10–12] and in the context of global IVIg shortages, identifying predictive factors of IVIg non-response has become increasingly important in order to optimise treatment selection. The authors of previous studies have suggested that response to IVIg improves in patients who are acetylcholine receptor antibody-positive (AChR+) [13, 14], in those with greater disease severity at baseline [13–16] and in patients with marked electrophysiological abnormalities [14, 15]. Conversely, patients with muscle-specific kinase antibody-positive (MuSK+) MG has been associated with poorer IVIg response [14, 17, 18].
The aim of the present study was to identify predictive factors of non-response to first-line IVIg in patients with generalised MG experiencing their first MC or exacerbation.
Methods
Study Design and Patients
This retrospective single-centre cohort study was conducted at the Hospices Civils de Lyon Groupement Hospitalier Est (HCL GHE) between February 2017 and July 2023.
We included adults (aged ≥ 18 years) with generalised autoimmune MG who experienced a first MC [4, 19] or exacerbation [20] and received first-line IVIg (1 or 2 g/kg). All patients were naïve to IVIg and PLEX therapy. Patients requiring subsequent PLEX due to IVIg failure were retained in the analysis, as they represented a refractory population. Patients presenting pure ocular MG were excluded.
This study was conducted in accordance with the Declaration of Helsinki and applicable national regulations on data protection. The study protocol was reviewed by the institutional ethics committee (Comité d’éthique, HCL number: 23-5460), which issued a favorable opinion. Patients were informed of the use of their anonymised data and did not express any opposition to the use of their data for research purposes.
Data Collection
Clinical, laboratory, electrophysiological and therapeutic data were retrospectively extracted from medical records. Laboratory parameters included C-reactive protein (CRP), neutrophil and lymphocyte counts, serum IgG levels at admission, antibody status (acetylcholine receptor [AChR], MuSK or double seronegative) and corresponding antibody titres. Electrophysiological data obtained from low-frequency repetitive nerve stimulation (LF-RNS) included the presence of a significant decrement (> 10%), the maximal decrement amplitude and the mean amplitude of pathological decrements. Thymectomy status, thymic histopathology and the interval between thymectomy and the exacerbation or MC were recorded. Data regarding prior and ongoing treatments, including corticosteroids, other immunosuppressive agents, and symptomatic therapies, were also collected.
Clinical status was retrospectively assessed at admission (day 0), discharge and at the 1-month follow-up visit. Muscle involvement was categorised as ocular, bulbar, axial, upper limb, or lower limb. The need for non-invasive or invasive ventilation (NIV/IV) and for nasogastric tube (NGT) placement was also documented.
Clinical severity and treatment response were evaluated using the Garches clinical score, a scale that is routinely employed in France for MG follow-up. This scale assesses muscle weakness in ocular (20 points), bulbar (30 points), axial (20 points), upper limb (15 points) and lower limb (15 points) domains, yielding a total score ranging from 0 (maximal weakness) to 100 (no weakness) [21].
The Myasthenia Gravis Foundation of America (MGFA) classification was also retrospectively determined for all patients, ranging from class I (isolated ocular weakness) to class V (requirement for mechanical ventilation).
Treatment response was defined as the maximal gain in Garches score following IVIg, calculated as the highest post-treatment score (at discharge or 1 month follow-up) minus the baseline score at admission. A clinically significant improvement was defined as an increase of at least 10 points in the Garches score. In patients who required add-on PLEX, response was evaluated relative to the Garches score immediately prior to PLEX initiation.
Statistical Analysis
Continuous variables were expressed as the median and interquartile range (IQR), and categorical variables as counts and percentage (%). Associations between all variables and IVIg response were assessed using linear regression models of Garches score gain, adjusted for baseline score. p-values < 0.05 were considered to be statistically significant. Analyses were performed using R software, version 4.0.3 ® Foundation for Statistical Computing, Vienna, Austria).
Results
Study Population
A total of 93 patients (55.9% male, 44.1% female; median age 68 [range 19–91] years) were included in the study. Median time from diagnosis to first IVIg was 0.7 (IQR 0.25–2.30) years. Of the 93 patients, 78 (83.9%) were anti-AchR+, seven (7.5%) were anti-MuSK+ and eight (8.6%) were double seronegative. The median Garches score at admission was 66.0 out of 100 (IQR 55.0–75.0), and 74.2% presented a MGFA score ≥ III.
Triggering factors were identified in 23 patients (24.7%), with the most prevalent being infectious diseases (7 patients, 7.5%) and use of contraindicated medications (6 patients, 6.5%). A minority of patients received a background treatment: 20 (21.5%) patients received corticosteroids, and 12 (12.9%) patients received other immunosuppressants. Prior thymectomy was identified in seven (7.5%) patients, with median time between surgery and first IVIg of 22 (IQR 5.0–184.0) months (Table 1).
Table 1.
Characteristics of patients enrolled in the study
| Characteristics | Population (N = 93) |
|---|---|
| Sex, N (%) | |
| - Male | 52 (55.9%) |
| - Female | 41 (44.1%) |
| Median age, years (IQR) | 68.00 (49–76) |
| Median BMI, kg/m2 (IQR) | 25.70 (23.30–28.80) |
| Auto-antibody, N (%) | |
| - AchR+ | 78 (83.9%) |
| - MuSK+ | 7 (7.5%) |
| - Double seronegative | 8 (8.6%) |
| Thymoma at time of first IVIg, n (%) | 14 (15.1%) |
| Thymic hyperplasia at time of first IVIg, n (%) | 9 (9.7%) |
| Thymic residue at time of first IVIg, n (%) | 11 (11.8%) |
| History of thymectomy at time of first IVIg, n (%) | 7 (7.5%) |
| Median time between MG diagnosis and first IVIg, years (IQR) | 0.70 (0.25–2.30) |
| Time between diagnosis and first IVIg < 3 months, n (%) | 30 (32.3%) |
| Myasthenia flare-up trigger, n (%) | |
| - All | 23 (24.7%) |
| - Infection | 7 (7.5%) |
| - Vaccination | 2 (2.2%) |
| - Medication | 6 (6.5%) |
| - Thymectomy | 2 (2.2%) |
| - Others | 8 (8.6%) |
| Treatments, n (%) | |
| - Corticosteroids | 20 (21.5%) |
| - Immunosuppressants | 12 (12.9%) |
| - Symptomatic treatments | 53 (57.0%) |
| Add-on PLEX after IVIg failure, n (%) | 5 (5.4%) |
| IVIg 1 g/kg; 2 g/kg, n (%) | 38 (40.9%), 55 (59.1%) |
| Median corticosteroids dosage at 1 month after IVIg, mg (IQR) | 20.00 (0.00–35.00) |
| Introduction of corticosteroids in the month following IVIg, n (%) | 49 (52.7%) |
| Median CRP, mg/L (IQR) | 2.20 (0.90–4.70) |
| Median neutrophils, G/L (IQR) | 4.65 (3.41–6.10) |
| Median lymphocytes, G/L (IQR) | 1.84 (1.38–2.32) |
| Median serum Ig assay, g/L (IQR) | 13.65 (11.88–16.00) |
| Median seric anti AchR levels, nmol/L (IQR) | 12.70 (5.60–17.62) |
| Median seric anti MuSK levels, UI/mL (IQR) | 18.94 (11.75–31.10) |
| Clinical involvement, n (%) | |
| - Upper limbs | 36 (38.7%) |
| - Lower limbs | 63 (67.7%) |
| - Bulbar | 74 (79.6%) |
| - Axial | 65 (69.9%) |
| - Ocular | 73 (78.5%) |
| - NIV/IV | 1 (1.1%) |
| - NGT | 5 (5.4%) |
| Significant RNS decrement, N (%) | 68 (82.9%) |
| Median mean RNS decrement, % (IQR) | 12.30 (6.92–23.40) |
| Median maximum RNS decrement, % (IQR) | 27.75 (16.30–47.75) |
| Median Garches score at day 0 (IQR) | 66.00 (55–75) |
| Median maximum Garches score after IVIg | 85.00 (75.00–94.00) |
| MGFA score at day 0, n (%) | |
| - MGFA I | 0 (0.0%) |
| - MGFA II | 24 (25.8) |
| - MFGA III | 50 (53.7%) |
| - MGFA IV | 18 (19.4%) |
| - MGFA V | 1 (1.1%) |
| MGFA score after IVIg, n (%) | |
| - MGFA I | 27 (29.0%) |
| - MGFA II | 47 (50.5%) |
| - MGFA III | 15 (16.2%) |
| - MGFA IV | 4 (4.3%) |
| - MGFA V | 0 (0.0%) |
AchR+ Acetylcholine antibodies, BMI body mass index, CRP C-reactive protein, Ig immunoglobulin, IQR interquartile range, IVIg intravenous immunoglobulins, MGFA Myasthenia Gravis Foundation of America classification, MuSK+ muscle specific kinase antibodies, NGT nasogastric tube, NIV/IV non-invasive ventilation/invasive ventilation, PLEX plasma exchange, RNS repetitive nerve stimulation
Response to IVIg Treatment
Median Garches score after treatment was 85.0 out of 100 (IQR 75.0–99.0) points, corresponding to a median gain of 15.0 (IQR 8.0–30.0) points. Clinical improvement occurred in 87.1% of patients, stability in 6.5% and worsening in 6.4% (Fig. 1).
Fig. 1.
Changes in Garches clinical score after intravenous immunoglobulin (IVIg) treatment. ‘Before’ refers to the Garches clinical score at admission prior to the initiation of IVIg rescue therapy (D0). ‘After’ refers to the best Garches clinical score after the initiation of IVIg treatment, i.e. between time of treatment initiation and evaluation at discharge or at the 1 month follow-up. Median Garches score was 66.0 (IQR 55.0–75.0) points at day 0 and 85.0 (IQR 75.0–95.0) points after IVIg initiation. Garches score improved in 81 (87.1%) patients, was stable in six (6.5%) patients and worsened in six (6.4%) patients. Median Garches gain was 15.0 (range − 6.0 to 74.0) points
A bulbar involvement was associated with a statistically significant greater response to IVIg, with mean difference of + 8.47 Garches score points (95% confidence interval [CI] 2.65–14.29, p = 0.005). No other clinical parameters influenced the response to IVIg.
Non-significant trends toward poorer response were observed in patients with an history of immunosuppressive treatment (mean gain of − 6.86 points, 95% CI − 13.92 to 0.20 points, p = 0.057), with prior thymectomy (mean gain of − 7.46 points, 95% CI − 16.35 to 1.43,, p = 0.099), with infectious trigger (mean gain of − 8.15 points, 95% CI − 17.02 to 0.71, p = 0.071) and requirement for NGT (mean gain of − 9.05 points, 95% CI − 19.74 to 1.64, p = 0.096).
Antibody status, IVIg dose, electrophysiological parameters, laboratory findings and associated thymic pathology did not significantly influence treatment response (see Electronic Supplementary Material [ESM] Appendix A; ESM Table 1).
Patients Requiring Add-On PLEX
Five patients required rescue PLEX after IVIg failure. All subsequently improved. This sub-population was exclusively composed of patients with AchR+ with recent disease onset (median time between MG onset and first IVIg of 1.0 [IQR 0.5–2.0] month compared to 4.0 [IQR 1.00–17.5] months in patients without PLEX). These patients had no prior immunosuppressive therapy and presented with greater baseline severity, with a median initial Garches score of 59.0 out of 100 (IQR 59.0–61.0) points compared to 68.0 out of 100 (IQR 55.0–77.2) points in patients without PLEX; the MGFA score was ≥ 3 for all five patients. The add-on PLEX population needed more NGT (2 out 5 patients; 40%) compared to the rest of the cohort (3 out of 88 patients; 3.4%). No consistent distinguishing predictive factor was identified in this sub-population (see ESM Appendix A; ESM Table 2).
Discussion
This study investigated predictive factors associated with response to first-line IVIg in a large cohort of patients with generalised MG presenting with a first exacerbation or MC. Identifying predictors of non-response could help clinicians consider alternative first-line strategies, such as plasma exchange, FcRn inhibitors or complement inhibitors, in selected patients [22, 23].
In this cohort, no independent predictor of IVIg non-response was identified. Only bulbar involvement was significantly associated with a greater response to IVIg. Several factors showed a non-significant trend toward poorer response, including prior use of immunosuppressive therapy (excluding corticosteroids), infectious trigger, previous thymectomy and need for NGT. Overall, IVIg was associated with a favourable clinical response in a population with heterogeneous disease characteristics.
The cohort accurately reflects the epidemiology of generalised MG, with approximately 80% patients with AchR+ and 7% patients with MuSK+ [24]. The median delay of 8 months between diagnosis and first IVIg suggests relatively recent disease onset, consistent with the low proportion of patients receiving background immunosuppressive therapy at the time of exacerbation or MC. Clinical severity was moderate overall: half of the patients were classified as MGFA III, bulbar involvement was present in 75% of patients and only one patient required mechanical ventilation, suggesting that most cases corresponded to exacerbations rather than full MC.
The observed trend toward poorer IVIg response in patients previously treated with non-steroidal immunosuppressants may reflect a subgroup with more refractory disease. This trend was not observed in patients treated with corticosteroids alone, which are widely recommended as first-line therapy and usually prescribed in standard rather than refractory cases.
A similar trend was observed in patients with prior thymectomy. Although thymectomy is known to reduce disease activity over time, the number of such patients in our cohort was small, and the delay between surgery and IVIg administration does not support a perioperative trigger effect. In current practice, IVIg is often administered before thymectomy in uncontrolled MG to prevent postoperative exacerbation [25], which may explain the limited number of patients who have been thymectomised receiving first-line IVIg in our study.
Exacerbations triggered by infection also showed a tendency toward poorer response. Infections, particularly respiratory infections, are well-recognised triggers of MC; however, their impact on IVIg responsiveness has not previously been reported. Although PLEX may represent an alternative in severe cases, its use can be challenging in patients with systemic infection, haemodynamic instability and vascular access concerns and in those limited by access to ICUs.
Patients requiring NGT also showed a trend toward poorer response, likely reflecting greater baseline severity, as these patients were frequently hospitalised in ICUs and sometimes required rescue PLEX following IVIg failure. Nevertheless, greater baseline clinical severity and very early disease onset (< 3 months) were not significantly associated with IVIg non-response in our supplementary analysis.
Interestingly, disease serotype (AchR+ , MuSK+ or double-seronegative) did not influence IVIg response. This contrasts with previous reports suggesting lower IVIg efficacy in MuSK+ MG, considered to be related to the predominance of IgG4 antibodies, which do not activate complement—one of the proposed mechanisms of IVIg action. However, the small number of patients with MuSK+ in our cohort limits definitive conclusions.
Electrophysiological parameters did not predict response to IVIg in our cohort. Although more pronounced abnormalities on repetitive nerve stimulation might theoretically reflect higher disease activity and therefore greater potential for improvement, electrophysiological–clinical dissociation is well recognised in MG [26, 27]. The authors of some previous studies have reported that the intensity of single fiber electromyography (SF-EMG) abnormalities was related to a better response to IVIg, [14, 15], but we did not study this possible association in the present study.
Bulbar involvement was the only significant predictor of better response to IVIg. However, this finding should be interpreted cautiously, as bulbar symptoms carry greater weight in the Garches clinical score (accounting for 30% of the total score), potentially amplifying measurable improvement in this domain.
PLEX may serve as a valuable alternative in patients with rapidly progressive MG, particularly when deterioration occurs within a short time frame (< 48 h) and baseline severity is high (MGFA IVb–V). PLEX often achieves a faster clinical response than IVIg, typically within a few days [28], making it especially relevant in these rapidly evolving scenarios. However, using PLEX after IVIg risks removing previously infused immunoglobulins and reducing efficacy. Although its use can be limited by vascular access, general context and ICU availability, early consideration of PLEX in this highly selected subset may provide the most efficient and timely therapeutic intervention for severe, rapidly progressing cases, and would allow the unnecessary use of IVIg to be avoided, in the context of recurrent supply shortages.
This study has a number of limitations, primarily related to its retrospective design and lack of standardised prospective evaluation. Although Garches and MGFA scores were available at baseline, post-treatment and the 1-month follow-up for all patients, missing data and small subgroup sizes may have limited statistical power. An additional limitation of this study relates to the potential confounding effect of concomitant corticosteroid therapy, either newly introduced or escalated following the first exacerbation or MC, and could have contributed to the observed improvement in Garches scores. Due to the retrospective design of the study, precise adjustment for these concomitant therapies was not feasible, and their potential influence on treatment response cannot be excluded. However, neither the presence of corticosteroids at the time of exacerbation (D0) nor the corticosteroid dose at the 1 month follow-up was associated with a significant impact on treatment response.
Conclusion
IVIg appears to be an effective first-line treatment for MG exacerbation and MC across a broad spectrum of clinical and biological profiles. No significant predictor of non-response was identified. While certain features showed a trend toward poorer response, these findings require confirmation in larger, prospective studies. Clinicians may nevertheless exercise caution in patients with immunosuppressive therapy, infectious triggers, prior thymectomy or severe bulbar dysfunction requiring NGT, in whom early consideration of alternative or rescue therapies may be appropriate.
Supplementary Information
Below is the link to the electronic supplementary material.
Acknowledgements
The authors acknowledge the Neuromuscular Reference Center at Hospices Civils de Lyon for institutional support, contribution to the care of patients and data collection. We thank the participants of the study.
Medical Writing, Editorial and Other Assistance
Medical writing assistance was provided by Dr Chanez Haouari, scientific medical writer at the Hospices Civils de Lyon. No external funding was received for this support.
Author Contributions
Antoine Gavoille performed the statistical analyses and critically revised the manuscript for important intellectual content. Antoine Pegat conceived the study and critically revised the manuscript for important intellectual content. Valérie Chamouard provided access to the treatment dispensing databases and contributed to data acquisition. Françoise Bouhour conceived the study and critically revised the manuscript for important intellectual content. Matthias Dion conceived the study, collected the data, drafted the manuscript and critically revised the manuscript for important intellectual content. All authors read and approved the final version of the manuscript.
Funding
No funding or sponsorship was received for this study or publication of this article. The Rapid Service Fee was funded by the authors.
Data Availability
The data that support the findings of this study are not publicly available due to data protection regulations and institutional restrictions but may be made available from the corresponding author upon reasonable request and with permission from the relevant data governance authorities.
Declarations
Conflict of Interest
Antoine Pegat has received consultancy fees, congress travel support and meeting participation fees from CSL Behring. Francoise Bouhour has received consultancy fees, congress travel support and meeting participation fees from CSL Behring. Valérie Chamouard has received consultancy fees, congress travel support and meeting participation fees from Novo Nordisk and Roche. Matthias Dion and Antoine Gavoille declare no conflicts of interest.
Ethical Approval
This study was conducted in accordance with the Declaration of Helsinki and applicable national regulations on data protection. The study protocol was reviewed by the institutional ethics committee (Comité d’éthique, HCL number 23-5460), which issued a favorable opinion. Patients were informed about the use of their anonymised data and did not express any opposition to its use for research purposes.
Contributor Information
Matthias Dion, Email: mat.dion@sfr.fr.
Françoise Bouhour, Email: francoise.bouhour@chu-lyon.fr.
References
- 1.Gilhus NE. Myasthenia gravis. N Engl J Med. 2016;375(26):2570–81. 10.1056/NEJMra1602678. [DOI] [PubMed] [Google Scholar]
- 2.Centre de références de pathologie neuromusculaire Paris Est. Protocole National de Diagnostic et de Soins (PNDS) myasthénie autoimmune. 2015. Accessed 5 Jan 2026. https://www.hassante.fr/upload/docs/application/pdf/2015-07/pnds_-_myasthenie_autoimmune.pdf
- 3.Thomas CE, Mayer SA, Gungor Y, et al. Myasthenic crisis: clinical features, mortality, complications, and risk factors for prolonged intubation. Neurology. 1997;48(5):1253–60. 10.1212/wnl.48.5.1253. [DOI] [PubMed] [Google Scholar]
- 4.Claytor B, Cho SM, Li Y. Myasthenic crisis. Muscle Nerve. 2023;68(1):8–19. 10.1002/mus.27832. [DOI] [PubMed] [Google Scholar]
- 5.Alhaidar MK, Abumurad S, Soliven B, Rezania K. Current treatment of myasthenia gravis. J Clin Med. 2022;11(6):1597. 10.3390/jcm11061597. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 6.Gajdos P, Chevret S, Clair B, Tranchant C, Chastang C. Clinical trial of plasma exchange and high-dose intravenous immunoglobulin in myasthenia gravis. Myasthenia Gravis Clinical Study Group. Ann Neurol. 1997;41(6):789–96. 10.1002/ana.410410615. [DOI] [PubMed] [Google Scholar]
- 7.Ballow M. Clinical and investigational considerations for the use of IGIV therapy. Am J Health Syst Pharm. 2005;62(16 Suppl 3):S12–18. 10.2146/ajhp050283. [DOI] [PubMed] [Google Scholar]
- 8.Bayry J, Dasgupta S, Misra N, et al. Intravenous immunoglobulin in autoimmune disorders: an insight into the immunoregulatory mechanisms. Int Immunopharmacol. 2006;6(4):528–34. 10.1016/j.intimp.2005.11.013. [DOI] [PubMed] [Google Scholar]
- 9.Stricker RB, Kwiatkowska BJ, Habis JA, Kiprov DD. Myasthenic crisis. Response to plasmapheresis following failure of intravenous gamma-globulin. Arch Neurol. 1993;50(8):837–40. 10.1001/archneur.1993.00540080046012. [DOI] [PubMed] [Google Scholar]
- 10.Guo R, Sun C, Huang X, et al. Efficacy and safety of efgartigimod versus intravenous immunoglobulin in early intervention of acetylcholine receptor antibody-positive impending myasthenic crisis: a retrospective cohort study. Neurotherapeutics. 2025;22(6):e00730. 10.1016/j.neurot.2025.e00730. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 11.Messina C, Basile L, Crescimanno G, et al. Prominent and fast response to Eculizumab in myasthenic crisis: the potential as rescue therapy in refractory myasthenia gravis. Neurol Sci. 2025;46(7):3299–302. 10.1007/s10072-025-08128-4. [DOI] [PubMed] [Google Scholar]
- 12.Rossini E, Morino S, Garibaldi M, et al. Emergent role of complement inhibitors in myasthenic crisis: understanding why, when and how. Clin Neurol Neurosurg. 2025;256:109011. 10.1016/j.clineuro.2025.109011. [DOI] [PubMed] [Google Scholar]
- 13.Barth D, Nabavi Nouri M, Ng E, Nwe P, Bril V. Comparison of IVIg and PLEX in patients with myasthenia gravis. Neurology. 2011;76(23):2017–23. 10.1212/WNL.0b013e31821e5505. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 14.Katzberg HD, Barnett C, Bril V. Predictors of response to immunomodulation in patients with myasthenia gravis. Muscle Nerve. 2012;45(5):648–52. 10.1002/mus.23236. [DOI] [PubMed] [Google Scholar]
- 15.Zinman L, Baryshnik D, Bril V. Surrogate therapeutic outcome measures in patients with myasthenia gravis. Muscle Nerve. 2008;37(2):172–6. 10.1002/mus.20908. [DOI] [PubMed] [Google Scholar]
- 16.Zinman L, Ng E, Bril V. IV immunoglobulin in patients with myasthenia gravis: a randomized controlled trial. Neurology. 2007;68(11):837–41. 10.1212/01.wnl.0000256698.69121.45. [DOI] [PubMed] [Google Scholar]
- 17.Pasnoor M, Wolfe GI, Nations S, et al. Clinical findings in MuSK-antibody positive myasthenia gravis: a U.S. experience. Muscle Nerve. 2010;41(3):370–4. 10.1002/mus.21533. [DOI] [PubMed] [Google Scholar]
- 18.Guptill JT, Sanders DB, Evoli A. Anti-MuSK antibody myasthenia gravis: clinical findings and response to treatment in two large cohorts. Muscle Nerve. 2011;44(1):36–40. 10.1002/mus.22006. [DOI] [PubMed] [Google Scholar]
- 19.Sanders DB, Wolfe GI, Benatar M, et al. International consensus guidance for management of myasthenia gravis: executive summary. Neurology. 2016;87(4):419–25. 10.1212/WNL.0000000000002790. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 20.Wiendl H, Abicht A, Chan A, et al. Guideline for the management of myasthenic syndromes. Ther Adv Neurol Disord. 2023;16:17562864231213240. 10.1177/17562864231213240. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 21.Sharshar T, Chevret S, Mazighi M, et al. Validity and reliability of two muscle strength scores commonly used as endpoints in assessing treatment of myasthenia gravis. J Neurol. 2000;247(4):286–90. 10.1007/s004150050585. [DOI] [PubMed] [Google Scholar]
- 22.Takahashi S, Kinno R. Management of myasthenic crisis and emerging roles of molecularly targeted therapies: a narrative review. Neurol Int. 2025;17(10):163. 10.3390/neurolint17100163. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 23.Tang P, He Y, Xiao K, Wang L. A systematic review of efgartigimod as an effective treatment for myasthenic crisis. Naunyn Schmiedebergs Arch Pharmacol. 2025;398(7):8287–94. 10.1007/s00210-025-03954-x. [DOI] [PubMed] [Google Scholar]
- 24.Carr AS, Cardwell CR, McCarron PO, McConville J. A systematic review of population based epidemiological studies in myasthenia gravis. BMC Neurol. 2010;10:46. 10.1186/1471-2377-10-46. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 25.Alipour-Faz A, Shojaei M, Peyvandi H, et al. A comparison between IVIG and plasma exchange as preparations before thymectomy in myasthenia gravis patients. Acta Neurol Belg. 2017;117(1):245–9. 10.1007/s13760-016-0689-z. [DOI] [PubMed] [Google Scholar]
- 26.Melzer N, Ruck T, Fuhr P, et al. Clinical features, pathogenesis, and treatment of myasthenia gravis: a supplement to the guidelines of the German Neurological Society. J Neurol. 2016;263(8):1473–94. 10.1007/s00415-016-8045-z. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 27.Tomschik M, Renaud E, Jäger F, et al. The diagnostic and prognostic utility of repetitive nerve stimulation in patients with myasthenia gravis. Sci Rep. 2023;13(1):2985. 10.1038/s41598-023-30154-5. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 28.Ghimire A, Kunwar B, Aryal B, et al. Assessing the comparative efficacy of plasmapheresis and intravenous immunoglobulin in myasthenia gravis treatment: a systematic review and meta-analysis. J Clin Neurosci. 2024;121:1–10. 10.1016/j.jocn.2024.01.025. [DOI] [PubMed] [Google Scholar]
Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Supplementary Materials
Data Availability Statement
The data that support the findings of this study are not publicly available due to data protection regulations and institutional restrictions but may be made available from the corresponding author upon reasonable request and with permission from the relevant data governance authorities.