Efgartigimod combined with steroids as a fast-acting therapy for myasthenic crisis: a case report.

BACKGROUND: Generalized myasthenia gravis (gMG) can be managed with acetylcholinesterase inhibitors (AChEis; e.g., pyridostigmine), corticosteroids, other immunosuppressive drugs (e.g., tacrolimus), and their combinations. Intravenous immunoglobulin (IVIg) or plasmapheresis (PLEX) may be administered if symptoms persist. PLEX and IVIg are also mainstays of treatment for myasthenic crisis. Recently, efgartigimod was approved in Japan for treating adults with gMG (irrespective of the antibody status) who do not have a sufficient response to corticosteroids and nonsteroidal immunosuppressive therapies. Efgartigimod is generally safe and well tolerated. However, since phase III trials of efgartigimod excluded those with myasthenic crisis, the efficacy of efgartigimod in treating myasthenic crisis is still unclear. Moreover, there are no reports that efgartigimod therapy can reduce the dose of corticosteroids needed to achieve a minimal manifestation status. CASE PRESENTATION: We report the case of a 70-yeat-old woman with gMG who developed a myasthenic crisis. After she was diagnosed with gMG, the patient had been treated with oral corticosteroids and tacrolimus for 1 year. However, she refused to continue taking the medication, and two weeks later, she developed ptosis, dysphagia and dyspnea. The patient was intubated and treated with efgartigimod in combination with steroid therapy, and she recovered without PLEX or IVIg. Afterward, when she experienced worsening of fatigue and increased levels of anti-acetylcholine receptor antibodies, efgartigimod therapy was effective. The patient achieved minimal manifestation status even after the reduction of corticosteroids and showed improvements in the Myasthenia Gravis Activities of Daily Living scales after 4 cycles of efgartigimod infusion. CONCLUSIONS: Our case suggests that efgartigimod can be an alternative drug for achieving minimal manifestation status in patients with myasthenic crisis. Considering its strong efficacy and safety, efgartigimod could be expanded to use as bridging therapy in the acute and chronic phases of gMG.

[Experience of using eculizumab in refractory myasthenia gravis]. / Opyt primeneniya ekulizumaba pri refrakternoi miastenii.

Myasthenia gravis (MG) is a chronic autoimmune disease mediated by autoreactive T- and B-cells and manifested by progressive pathological muscle weakness and fatigue. Traditional immunomodulatory treatment does not always lead to the clinical picture significant improvement, despite adequate dosage and duration of use. Refractory myasthenia gravis requires new therapeutic approaches development and implementation. The range of target innovative agents in refractory MG includes monoclonal antibodies, which act directly on individual components of the complement system. Based on the results of randomized controlled trials, data on the effectiveness and safety of eculizumab, which inhibits the C5 component of the complement system, is presented. We present our own clinical experience of using eculizumab in a young woman with refractory generalized AChR-positive MG with a rapid decrease in the severity of symptoms to a minimum level and restoration of ability to work, and the absence of adverse events during therapy.

Serological indication of chronic inflammatory demyelinating polyneuropathy as an extrahepatic manifestation of hepatitis E virus infection.

Guillain-Barré syndrome and neuralgic amyotrophy have been associated with hepatitis E virus (HEV) genotype 3 infections, while myasthenia gravis (MG) has been associated with HEV genotype 4 infections. However, whether chronic inflammatory demyelinating polyneuropathy (CIDP) is associated with HEV infections has not been conclusively clarified yet. 102 CIDP patients, 102 age- and sex-matched blood donors, 61 peripheral neuropathy patients (non-CIDP patients), and 26 MG patients were tested for HEV and anti-HEV IgM and IgG. Sixty-five of the 102 (64%) CIDP patients tested positive for anti-HEV IgG and one (1%) for anti-HEV IgM. No other patient tested positive for ati-HEV IgM. In the subgroup of CIDP patients with initial diagnosis (without previous IVIG treatment), 30/54 (56%) tested positive for anti-HEV IgG. Anti-HEV rates were significantly lower in blood donors (28%), non-CIDP peripheral neuropathy patients (20%), and MG patients (12%). No subject tested positive for HEV viremia. CSF tested negative for in 61 CIDP patients (54 patients with primary diagnosis). The development of CIDP but not non-CIDP polyneuropathy may be triggered by HEV exposure in an HEV genotype 3 endemic region. The increased anti-HEV seroprevalence in CIDP patients is not a consequence of IVIG therapy.

Efgartigimod-associated Kaposi's varicelliform eruption and herpetic conjunctivitis in a patient with seropositive ocular myasthenia gravis: a case report and review.

Background: Efgartigimod (Efgartigimod alpha fcab, Vyvgart™) is a pioneering neonatal Fc receptor (FcRn) antagonist for the treatment of severe autoimmune diseases mediated by pathogenic immunoglobulin G (IgG) autoantibodies, including myasthenia gravis (MG). It is a well-tolerated drug with minor side effects, such as headache and upper respiratory (lung) and urinary tract infections. Here, we present a case of Kaposi's varicelliform eruption (KVE) and herpetic conjunctivitis related to efgartigimod in a 60-year-old patient with ocular MG (OMG). Case description: A 60-year-old Chinese male suffered from acetylcholine receptor antibody positive (AChR Ab+) OMG for 8 years. During this period, he underwent first-line treatment with systemic corticosteroids, cyclosporine, cyclophosphamide, and so on, but had poor symptom improvement. On the recommendation of his attending neurologist, he received one cycle of intravenous efgartigimod (10mg/kg, once weekly for 4 weeks). The patient experienced fever, widespread painful blisters, and edema on the face on the third day after his last intravenous infusion. The patient also complained of increased secretions and a foreign body sensation in both eyes. Laboratory tests confirmed infection with herpes simplex virus (HSV). A diagnosis of efgartigimod-associated KVE and herpetic conjunctivitis was made. After intravenous administration (5mg/kg, 3 times a day, every 8 hours) for 10 days, the patient was cured without residual complications. Conclusions: This case is the first report of a patient with KVE and herpetic conjunctivitis related to efgartigimod in PubMed. This is rare and unusual. Clinicians should be alert to the rare symptoms related to efgartigimod.

Myasthenia gravis.

Myasthenia gravis (MG) is a rare neuromuscular junction disorder that is characterized by fatigable weakness of muscles. People with MG experience various clinical manifestations based on the muscles involved. MG can be autoimmune, paraneoplastic, congenital, medication-related, or transient in the neonatal period due to the passive placental transfer of antibodies from mothers with MG. Acetylcholine receptor antibodies are seen in the majority of patients with MG. However, other antibodies have been discovered in the last 20 years, including muscle-specific tyrosine kinase (MuSK) and lipoprotein-related peptide 4 (LRP4), and are now available through commercial testing. More recently, a handful of other antibodies have been associated with MG; however, they are not presently available for routine testing. A disease classification system has been developed by the Myasthenia Gravis Foundation of America (MGFA) and is commonly used worldwide. A number of objective and subjective outcome measures have been developed and validated over the years and have been proven useful for both clinical and research purposes, serving as primary and secondary outcome measures in most clinical trials. A growing number of therapies are available for both acute and chronic management of MG, with several new mechanistic approaches under investigation. An international consensus guidance for the management of MG was first published in 2016 and updated in 2020.

Neuromuscular junction disorders: mimics and chameleons.

Neuromuscular junction (NMJ) disorders represent a heterogenous group of acquired and congenital disorders that present in variable and distinctive ways. The diagnosis is typically reached through a combination of clinical, serological, pharmacological and electrophysiological evaluation. While the diagnosis can be fairly straightforward in some cases, the overlap with other neurological disorders can make diagnosis challenging, particularly in pure ocular presentations and in seronegative patients. The over-reliance on serological tests and electrophysiological evaluation in isolation can lead to misdiagnosis. In this article, we provide an overview of the NMJ disorders, discuss red flags for the key differential diagnoses (mimics) and report the atypical ways in which NMJ disorders may present (chameleons).

Efgartigimod as a fast-acting add-on therapy in manifest and impending myasthenic crisis: A single-center case series.

Efgartigimod was the first-in-class neonatal Fc receptor antagonist approved for the treatment of acetylcholine receptor antibody positive (AChR+), Myasthenia Gravis Foundation of America (MGFA) Class II-IV generalized myasthenia gravis (gMG) patients. As a novel therapy, the clinical experiences are still lacking, especially for the use of efgartigimod in manifest and impending myasthenic crisis (IMC). We reported three AChR+, gMG patients, two with myasthenic crisis (MC) and one with IMC, treated with efgartigimod. MGFA class, MG-Activity of Daily Living score (MG-ADL), Quantitative MG score (QMG), and Muscle Research Council sum score (MRC), concentration of anti-AChR antibody, IgG, globulin, and albumin, subsets of T and B lymphocyte were evaluated or measured before, during and after efgartigimod treatment. All patients showed fast and robust response to efgartigimod with marked improvement in MGFA, MG-ADL, QMG, and MRC scores. Patient 1 did not respond effectively to IVIg but was successfully rescued by add-on efgartigimod. She extubated at 7 days after the first infusion and got rid of NIV after 14-days treatment. Patient 2 and patient 3 directly used efgartigimod when symptoms were not ameliorated by adjusting of oral drugs. Patient 2 wean from BiPAP at seven days after the first infusion. Patient 3 in IMC status, overcame the severe dysphagia at three days after the first infusion. Clinical symptoms continued to improve 1-2 weeks after discharge. Concentration of anti-AChR antibody, IgG and globulin were remarkably reduced by efgartigimod treatment. Our study supported that efgartigimod could act as a fast-acting rescue therapy for patients with MC or IMC. Larger studies from multicenter are required to provide further evidence.

Worsening of myasthenic symptoms associated with statins.

INTRODUCTION/AIMS: The common presentations of statin intolerance are muscle-specific symptoms. Although statins are one type of drug reported to cause myasthenic worsening, myasthenic worsening has not been recognized as statin intolerance. The purpose of the present study is to investigate in a large cohort the safety profiles of statins in patients with myasthenia gravis (MG). METHODS: A total of 1710 consecutive patients with MG who visited sites associated with the Japan MG registry 2021 group between April and October 2021 were reviewed. Statin-associated myasthenic worsening was defined as worsening of any myasthenic symptoms on statin use and improvement of the symptom by stopping the statin or by undertaking additional treatment with patient and doctor confirmation. RESULTS: Among the 400 patients who used statins, 8 (2%) patients experienced statin intolerance and 6 (1.5%) patients experienced myasthenic worsening. No patients developed MG on the statin. Ptosis was a main symptom of myasthenic worsening in 4 (67%) patients. Atorvastatin was used in all patients with statin-associated myasthenic worsening. The symptoms of statin intolerance and statin-associated myasthenic worsening were improved within 2 months and 3 months, respectively, in all patients by cessation of statin use. DISCUSSION: Regarding statin-associated myasthenic worsening, prevalence was low, and severity was mild; with cessation of statin use, symptoms improved within a few months, and outcomes were generally good. Although statins can be used in MG patients with little concern, statin-associated myasthenic worsening should be noted in addition to the classical statin intolerance associated with statin use.

[Thoracoscopic thymectomy for myasthenia gravis and non-invasive thymoma after COVID-19 pneumonia]. / Torakoskopicheskaya timektomiya pri miastenii i neinvazivnoi timome posle COVID-19 pnevmonii.

Modern guidelines have identified thoracoscopic thymectomy as a preferable option for myasthenia gravis and non-invasive thymoma. In the era of the new coronavirus infection, it is relevant to develop protocols for anesthetic and perioperative support of patients undergoing thymectomy for myasthenia gravis after COVID-associated pneumonia (CAP). We present the results of thoracoscopic thymectomies in patients after CAP. Multidisciplinary team should determine therapeutic support, the need for plasmapheresis and thymectomy. Plasmapheresis and glucocorticosteroids are effective in addition to anticholinesterase therapy at the stages of perioperative support for correction of neurological status in patients with myasthenia combined with chronic obstructive pulmonary disease and pulmonary hypertension. Outpatient direct anticoagulants are advisable considering the need for prolonged postoperative prevention of thrombotic events.

Pembrolizumab-induced myasthenia gravis: Two patients' experiences.

Checkpoint inhibitors are increasingly used to treat patients with gynecologic malignancies and can cause rare and unusual side effects, also known as immunotoxicities, that are rarely observed in patients receiving traditional immunotherapy. If these are not identified and treated, they can cause disability and even death for patients undergoing treatment. This report describes the range of pembrolizumab-induced myasthenia gravis (MG) immunotoxicity through two cases. The first patient is an 85-year-old woman with recurrent vulvar carcinoma who completed two cycles of pembrolizumab. She had a severe presentation leading to respiratory failure. The second patient is an 80-year-old woman with recurrent serous endometrial carcinoma who developed isolated ocular myasthenia after her second cycle of pembrolizumab. The symptoms and physical examination findings described here illustrate the breadth of symptom severity associated with pembrolizumab-induced MG and importance of early identification and treatment to minimize symptoms and improve outcomes.

Weighted gene coexpression network analysis and machine learning for the determination of tfh cell and B cell infiltrating biomarkers in thymoma-associated myasthenia gravis.

Patients with thymoma (THYM)-associated myasthenia gravis (MG) typically have a poor prognosis and recurring illness. This study aimed to discover important biomarkers associated with immune cell infiltration and THYM-associated MG (THYM-MG) development. Gene expression microarray data were downloaded from The Cancer Genome Atlas website (TCGA) and Gene Expression Omnibus (GEO). A total of 102 differentially expressed genes were investigated. According to the immune infiltration data, the distribution of Tfh cells, B cells, and CD4 T cells differed significantly between the THYM-MG and THYM-NMG groups. WGCNA derived 25 coexpression modules; one hub module (the blue module) strongly correlated with Tfh cells. Combining differential genes revealed 21 intersecting genes. LASSO analysis subsequently revealed 16 hub genes as potential THYM-MG biomarkers. ROC curve analysis of the predictive model revealed moderate diagnostic value. The association between the 16 hub genes and infiltrating immune cells was further evaluated in TIMER2.0 and the validation dataset. Draggability analysis identified the therapeutic target genes PTGS2 and ALB, along with significant drugs including Firocoxib, Alclofenac, Pyridostigmine, and Stavudine. This was validated through MD simulation, PCA, and MM-GBSA analyses. The interaction between numerous activated B cells and follicular helper T cells is closely associated with THYM-MG pathogenesis from a bioinformatics perspective. Hub genes (including SP6, SCUBE3, B3GNT7, and MAGEL2) may be downregulated in immune cells in THYM-MG and associated with progression.

Efgartigimod Successfully Ameliorated Acute Exacerbation of Myasthenia Gravis with Anti-muscle-specific Kinase Antibodies.

We herein report two patients with anti-muscle-specific kinase (MuSK) antibody-positive myasthenia gravis who experienced rapid deterioration of weakness, particularly respiratory muscle weakness, necessitating non-invasive positive pressure ventilation (NIPPV) and were treated with efgartigimod. After treatment initiation, a rapid reduction in IgG levels and recovery from clinical symptoms were observed. NIPPV was no longer required two to three weeks after the first infusion of efgartigimod. These findings suggest that the reduction of IgG levels using efgartigimod is a good treatment option in patients with myasthenia gravis positive for anti-MuSK antibodies, even during the acute phase of the disease.

HSCT for stiff person syndrome and myasthenia gravis.

Recent advances in neuroimmunology have shed light on the pathogenic mechanisms underlying rare neuroimmunologic conditions such as myasthenia gravis (MG) and stiff person syndrome (SPS). Despite the rarity of these conditions, their complex manifestations and potential for irreversible disability necessitate effective therapeutic strategies. This chapter reviews the current understanding of the safety and efficacy of hematopoietic stem cell transplantation (HSCT) in MG and SPS. Several case reports and retrospective studies have demonstrated promising outcomes following HSCT in refractory MG and SPS, with significant clinical improvement and even discontinuation of chronic immunomodulatory therapy in some cases. Furthermore, HSCT may offer insights into the underlying pathophysiologic mechanisms of these conditions, particularly the role of cellular immunity. Although more research is needed to fully understand the impact of HSCT on disease pathology and outcomes, current evidence suggests that HSCT could be a valuable therapeutic option for patients with refractory MG and SPS.

Visualization and characterization of complement activation in acetylcholine receptor antibody seropositive myasthenia gravis.

INTRODUCTION/AIMS: There are no blood biomarkers to monitor treatment effects in myasthenia gravis (MG) or studies visualizing the acetylcholine receptor (AChR) antibody-induced membrane attack complex (MAC) at the human muscle membrane. This study aimed to compare levels of complement activation products and native complement components in MG patients and healthy controls (HCs) and to model the AChR antibody-mediated attacks in human muscle cells. METHODS: We assessed the complement components and activation product levels with enzyme-linked immunosorbent assay and magnetic bead-based sandwich assays in plasma and sera of 23 MG patients and matched HCs. Receiver operator characteristic (ROC) curve analysis evaluated the diagnostic accuracy. Complement levels were correlated with the myasthenia gravis composite (MGC) scores. AChR+ MG modeling in human muscle cells used sera from nine MG patients and three HCs. RESULTS: MG patients had significantly higher plasma levels of C3a (p < .0001), C5 (p = .0003), and soluble C5b-9 (sC5b-9; p < .0001) than HCs. The ROC curve analysis showed a clear separation between MG patients and HCs for plasma C3a (AUC = 0.9720; p < .0001) and sC5b-9 (AUC = 0.8917, p < .0001). MG patients had higher levels of plasma complement Factor I (FI; p = .0002) and lower properdin levels (p < .0001). The MGC had moderate correlations with plasma Factor B (FB), FI, and Factor H. AChR+ MG patient sera triggered the deposition of MAC and reduced AChRs. DISCUSSION: We suggest validating plasma C3a and sC5b-9 as blood biomarkers for complement activation in MG. Further, the in vitro study allowed visualization of MAC deposition after applying AChR+ MG sera on human muscle cells.

The speed of completion of the decremental responses on repetitive nerve stimulation.

Objective: It is generally believed that the decremental response in repetitive nerve stimulation (RNS) stabilizes at the fourth or fifth response. We have a preliminary impression that the decremental response approaches a plateau earlier in proximal muscles than in distal muscles. We investigated the speed of the completion of the decremental response in different muscles. Methods: The "decrement completion ratio (DCR)" in the second or third response (DCR2 or DCR3) was defined as the ratio of the decremental percentage of the second or third response to that of the fourth response. Patients showing more than 10% decremental response both in the abductor pollicis (APB) and deltoid muscles were retrospectively extracted from our EMG database. The DCR2 and DCR3 were compared between two muscles in patients with myasthenia gravis (MG) and amyotrophic lateral sclerosis (ALS). Results: Identified subjects consisted of 11patients with MG and 11 patients with ALS. Multiple regression analysis revealed that only the difference of muscle influenced on DCR2 and DCR3, with no contribution from the different disorder (MG or ALS) or the initial amplitude of the compound muscle action potential (CMAP). Both DCR2 and DCR3 were significantly higher in deltoid than in APB. In ALS, the normalized CMAP amplitude was not different between APB and deltoid whereas the decremental percentage was significantly higher in deltoid, suggesting a lower safety factor of the neuromuscular transmission in proximal muscles. Conclusions: The decremental response completed more rapidly in deltoid than in APB which may be related to the lower safety factor also documented by this study. Significance: Unexpected early completion of the decrement such as at the second response in RNS is not a technical error but may be an extreme of the rapid completion in deltoid, a proximal muscle.

Diurnal cognitive functioning in patients with myasthenia gravis with the role of chronotype and depression: a pilot study.

Purpose: Myasthenia gravis (MG) is an autoimmune disease manifested by fatigue and weakness of the skeletal muscles. Recent research has indicated that MG patients perform significantly worse than healthy controls in cognitive domains such as attention, verbal fluency, visual learning, and memory. This study aimed to investigate the diurnal fluctuations in cognitive performance in patients with myasthenia gravis in relation to selected clinical and socioeconomic parameters of the disease course, along with the role of chronotype and depression. Methods: The participants were recruited from a neurology outpatient clinic. Patients' cognitive functions were assessed twice: in the morning and the evening of the same day. Neuropsychological diagnosis included attention, memory, executive, verbal, and visuospatial abilities. Mood was measured with the Beck Depression Inventory-II and Positive and Negative Affect Schedule. The Morningness-Eveningness Questionnaire was used to examine chronotype. Results: The analyses performed showed no significant differences between subjects and within subjects, apart from semantic fluency. Patients receiving antidepressant treatment obtained better results on attention and working memory tasks. Conclusions: The data obtained show that diurnal neuropsychological performance in MG patients is associated with depression. Routine assessment and treatment of mood disorders could significantly improve cognitive functioning in myasthenia gravis patients.

FOSL1-mediated LINC01566 negatively regulates CD4+ T-cell activation in myasthenia gravis.

BACKGROUND: Myasthenia gravis (MG) is an autoimmune disease characterized by pathogenic antibodies that target structures of the neuromuscular junction. The evidence suggests that the regulation of long noncoding RNAs (lncRNAs) that is mediated by transcription factors (TFs) plays a key role in the pathophysiology of MG. Nevertheless, the detailed molecular mechanisms of lncRNAs in MG remain largely undetermined. METHODS: Using microarray analysis, we analyzed the lncRNA levels in MG. By bioinformatics analysis, LINC01566 was found to potentially play an important role in MG. First, qRT‒PCR was performed to verify the LINC1566 expressions in MG patients. Then, fluorescence in situ hybridization was conducted to determine the localization of LINC01566 in CD4 + T cells. Finally, the impact of LINC01566 knockdown or overexpression on CD4 + T-cell function was also analyzed using flow cytometry and CCK-8 assay. A dual-luciferase reporter assay was used to validate the binding of the TF FOSL1 to the LINC01566 promoter. RESULTS: Based on the lncRNA microarray and differential expression analyses, we identified 563 differentially expressed (DE) lncRNAs, 450 DE mRNAs and 19 DE TFs in MG. We then constructed a lncRNA-TF-mRNA network. Through network analysis, we found that LINC01566 may play a crucial role in MG by regulating T-cell-related pathways. Further experiments indicated that LINC01566 is expressed at low levels in MG patients. Functionally, LINC01566 is primarily distributed in the nucleus and can facilitate CD4 + T-cell apoptosis and inhibit cell proliferation. Mechanistically, we hypothesized that LINC01566 may negatively regulate the expressions of DUSP3, CCR2, FADD, SIRPB1, LGALS3 and SIRPB1, which are involved in the T-cell activation pathway, to further influence the cellular proliferation and apoptosis in MG. Moreover, we found that the effect of LINC01566 on CD4 + T cells in MG was mediated by the TF FOSL1, and in vitro experiments indicated that FOSL1 can bind to the promoter region of LINC01566. CONCLUSIONS: In summary, our research revealed the protective roles of LINC01566 in clinical samples and cellular experiments, illustrating the potential roles and mechanism by which FOSL1/LINC01566 negatively regulates CD4 + T-cell activation in MG.

Frequency and severity of autonomic dysfunction assessed by objective hemodynamic responses and patient-reported symptoms in individuals with myasthenia gravis.

Introduction: Myasthenia gravis (MG), a rare autoimmune disorder, poses diagnostic and management challenges, with increasing incidence in Europe and significant impact on patient quality of life. Despite prevalent autonomic symptoms, comprehensive assessments integrating subjective and objective measures are lacking. We aimed to investigate the prevalence and severity of autonomic dysfunction in patients with MG and healthy controls (HCs). Materials and methods: We used beat-to-beat hemodynamic responses during standardized autonomic function tests (AFTs) and the Composite Autonomic Symptom Score 31 (COMPASS-31) questionnaire. Study participants including, 53 patients with MG and 30 age- and sex matched HCs underwent standardized cardiovascular AFTs and completed the COMPASS-31 questionnaire. Patients were categorized into Non-CAN and CAN groups based on their Cardiovascular Autonomic Neuropathy (CAN) status, as evaluated using the Composite Autonomic Scoring Scale (CASS). During the AFTs, cardiovascular parameters including heart rate, systolic blood pressure (BP), diastolic BP, mean BP, stroke volume (SV), cardiac output (CO), and total peripheral resistance (TPR) were measured. Results: Twenty patients with MG (38%) exhibited mild CAN (CASS ≥2) with a median total CASS score of 1.00 and CASS 0.00 in HCs. Adrenergic impairment was observed in 27 patients (52%), with 13 patients (24.5%) exhibiting longer pressure recovery time after Valsalva maneuver (VM). Cardiovagal impairment was evident in 71% of patients, with abnormal results observed in 39.6% for the deep breathing test and 56.6% for the VM. CAN MG showed worse scores than HCs for the total COMPASS-31 (p < 0.001), orthostatic (OI) (p < 0.001), secretomotor (p = 0.004), and pupillomotor domains (p = 0.004). Total COMPASS-31 and OI scores were correlated with worse disease outcomes (disease duration, severity), hemodynamic parameter changes (SV, CO, TPR) during phase II late of VM, and with changes (Δtilt-supine) in Δsystolic BP, Δdiastolic BP, Δmean BP, ΔTPR during head-up-tilt test, but not with CASS score. Conclusion: Our findings demonstrate mild cardiovascular autonomic impairment in adrenergic and cardiovagal domains in patients with MG. Additionally, patient-reported autonomic symptoms correlated with hemodynamic changes during AFTs and worse disease outcomes and not with the grade of autonomic abnormalities. Incorporating beat-to-beat hemodynamics during AFTs may offer further insights for characterizing orthostatic intolerance symptoms in MG group.

Clinical features of thymoma with and without myasthenia gravis.

Objective: To explore the clinical features of thymoma with and without myasthenia gravis (MG). Methods: This was a retrospective study. Two hundred and thirty-three patients with mediastinal masses who were initially diagnosed in People's Hospital of Shijiazhuang, China, between January 2014 and June 2022 and had complete clinical data and underwent surgical treatment at People's Hospital of Shijiazhuang were retrospectively analyzed. Result: The age of patients with thymoma alone was significantly older than that of thymoma patients complicated with MG. The number of female patients was slightly more than males for both groups. Proportions of type A, AB, B1, B2, and B3 thymomas in Group-A were 0.77, 11.54, 11.51, 33.85, and 31.54%, respectively, and the proportions in Group-B were 9.68, 22.58, 12.90, 32.26, and 22.58%. The size of tumors in patients with thymoma alone was larger than that of patients with thymoma complicated with MG. The proportion of patients with tumor size of more than 10 cm in the thymoma alone group was significantly higher than that in the MG group. There were no relapses in patients with type A disease and relapses were noted in a few patients with type B1, B2 and B3 diseases. The same survival rates were reported for the two groups. Conclusion: MG rarely occurs in type A and type C diseases. The prognosis of thymoma with MG is similar to that of thymoma alone. The main causes of death may be myasthenia crisis in thymoma patients with MG and advanced tumor stage in patients with thymoma alone.