Cardiac implications in myasthenia gravis

This editorial aimed to consolidate the current evidence in literature on the association between myasthenia gravis(MG)and cardiac involvement,focusing on the impact of thymoma,antistriational antibodies,and late-onset MG.Additionally,the study aimed to explore the influence of genetic differences among populations on the association with cardiac disease.We conducted a review of existing literature in PubMed and Google Scholar to find relevant studies on cardiac involvement in MG.We created search criteria using a combination of free text words,including MG,antistriational antibodies,thymectomy,cardiomyopathy,myocarditis,arrhythmias,autonomic dysfunction.Relevant articles published in English language were analyzed and incorporated.The findings indicate a strong association between thymoma,myasthenic crisis,antistriational antibodies,and late-onset MG with cardiac involvement.The study also revealed that genetic differences among populations influence the risk of cardiac disease and electrocardiography(ECG)abnormalities in MG patients.Autonomic dysfunctions altered cardiac autonomic response and increased susceptibility to arrhythmias and sudden cardiac death in MG patients.The study supports the significance of thymoma,antistriational antibodies,and late-onset MG as key factors associated with cardiac involvement in MG patients.It emphasizes the importance of ECG as the initial test in managing MG patients,particularly in the perioperative period,to identify and genetic testing if needed to address their cardiac risk effectively.

Perioperative cardiac risks in myasthenia gravis

Myasthenia gravis(MG)is an autoimmune disorder that affects the neuromuscular junction.The primary pathology in MG involves the presence of autoantibodies to acetylcholine receptors(AChRs),which results in qualitative and quantitative reductions in the availability of functional AChRs.Cardiac muscles are also affected,resulting in various perioperative cardiac complications.Antistriational antibodies are commonly reported in MG cases with cardiac involvement.In the presence of thymoma,the prevalence of cardiac manifestations in patients with MG increases to approximately 10%-15%.Cardiac involvement in MG may range from asymptomatic electrocardiogram changes to ventricular tachycardia,myocarditis,conduction disorders,heart failure,and sudden death.Increased incidence of atrial fibrillation,ventricular and supraventricular extra systoles,and prolonged QTc have also been reported in patients with MG.Clinicians should consider the evaluation of autonomic dysfunction and risk of cardiovascular disease in patients with MG.

Possible implications of dysregulated nicotinic acetylcholine receptor diffusion and nanocluster formation in myasthenia gravis

Myasthenia gravis is a rare and invalidating disease affecting the neuromuscular junction of voluntary muscles.The classical form of this autoimmune disease is characterized by the presence of antibodies against the most abundant protein in the neuromuscular junction,the nicotinic acetylcholine receptor.Other variants of the disease involve autoimmune attack of non-receptor scaffolding proteins or enzymes essential for building or maintaining the integrity of this peripheral synapse.This review summarizes the participation of the above proteins in building the neuromuscular junction and the destruction of this cholinergic synapse by autoimmune aggression in myasthenia gravis.The review also covers the application of a powerful biophysical technique,superresolution optical microscopy,to image the nicotinic receptor in live cells and follow its motional dynamics.The hypothesis is entertained that anomalous nanocluster formation by antibody crosslinking may lead to accelerated endocytic internalization and elevated turnover of the receptor,as observed in myasthenia gravis.

“Warming yang and invigorating qi” acupuncture alters acetylcholine receptor expression in the neuromuscular junction of rats with experimental autoimmune myasthenia gravis

Myasthenia gravis is an autoimmune disorder in which antibodies have been shown to form against the nicotinic acetylcholine nicotinic postsynaptic receptors located at the neuromuscular junction.＂Warming yang and invigorating qi＂ acupuncture treatment has been shown to reduce serum inflammatory cytokine expression and increase transforming growth factor beta expression in rats with experimental autoimmune myasthenia gravis.However,few studies have addressed the effects of this type of acupuncture on the acetylcholine receptors at the neuromuscular junction.Here,we used confocal laser scanning microscopy to examine the area and density of immunoreactivity for an antibody to the nicotinic acetylcholine receptor at the neuromuscular junction in the phrenic nerve of rats with experimental autoimmune myasthenia gravis following ＂warming yang and invigorating qi＂ acupuncture therapy.Needles were inserted at acupressure points Shousanli（LI10）,Zusanli（ST36）,Pishu（BL20）,and Shenshu（BL23） once daily for 7 consecutive days.The treatment was repeated after 1 day of rest.We found that area and the integrated optical density of the immunoreactivity for the acetylcholine receptor at the neuromuscular junction of the phrenic nerve was significantly increased following acupuncture treatment.This outcome of the acupuncture therapy was similar to that of the cholinesterase inhibitor pyridostigmine bromide.These findings suggest that ＂warming yang and invigorating qi＂ acupuncture treatment increases acetylcholine receptor expression at the neuromuscular junction in a rat model of autoimmune myasthenia gravis.

Computer Aided Study of Mechanism of Immunoadsorbent for Myasthenia Gravis

Myasthenia Gravis (MG) is an organ specific autoimmune disease mediated by autoantibodies (AChR Ab) against the acetylcholine receptor (AChR). Literature reported that the AChR Ab can be removed by absorbent linked with tryptophan. It was studied in detail in our lab. With the aid of computer, we docked some ligands into AChR Ab, and the results from scores of docking under different generations showed that there was no specific binding between tryptophan and scFv fragment just as the binding between antigen-antibody. The interaction between Trp and immunoglobulin was a broad-spectrum binding.

Enzyme-linked Immunosorbent Assay for Detection of Anti-idiotype Antibodies to Antibodies to Ligand of Nicotinic Acetylcholine Receptor in Sera of Patients with Myasthenia Gravis

Anti-bungarotoxin anti-serum,which has the internal image of nicotinicacetylcholine receptor,was used as a tool to measure anti-idiotypic antibodies toantibodies to Iigand of nicotinic acctylcholine receptor in scra from 81 patients withmyasthenia gravis.Enzyme-linked immunosorbcnt assay was adopted.Thc positive ratewas 46.9%(38/81).The specific cross inhibitory test with nicotinic acetylcholinereceptor was positive.Anti-idiotype antibodies to antibodies to ligand of nicotinicacetylcholine receptor in sera of different types of myasthenia gravis patients classified ac-cording to modified Osserman’s standard and myasthenia gravis patients with or withoutthymoma were comparcd in this study and the role of anti-idiotype antibodies toantibodies to Iigand of nicotinic acctylcholinc receptor in the immunity of myasthcniagravis and the possibility of thcrapeutic use of anti-idiotype antibodies arc discussed.

Experimental Study on Induction of Tolerance to Experimental Autoimmune Myasthenia Gravis by Immature Dendritic Cells

Summary: To investigate the effect of immature dendritic cells (iDCs) on experimental autoimmune myasthenia gravis (MG), iDCs were generated in low dose of GM-CSF, and then they were pulsed with acetylcholine receptor (AchR) and transferred to allogeneic rats. After 3 weeks, all rats were immunized with AchR and complete Freund's adjuvant (CFA) and observed for the corresponding indices of MG for 7 weeks. Our results showed that compared with mature DCs (mDCs) generated at high dose of GM-CSF plus additional stimulation by lipopolysaccharide, iDCs expressed significantly lower levels of MHC-Ⅱ, CD80 and CD86, and their ability to uptake FITC-Dextran was stronger but the ability of stimulating proliferation of allogeneic T cells were weaker. Like controls, after immunization, all rats transferred with iDCs, mDCs and AchR-pulsed mDCs showed typical symptoms in 4 to 7 weeks. The amplitude of electromyogram wave dropped obviously, the level of serum AchRab increased and neuromuscular junction showed typical damage of MG. In contrast, no conspicuous changes were noted in rats transferred with AchR-pulsed iDCs. The results suggest that iDCs could be generated by inducing bone marrow precursors in low dose of GM-CSF, AchR-pulsed iDCs could induce tolerance of EAMG. The dysfunction of DCs may play an important role in the initiation and maintenance of normal immune response in MG.

Effects of intravascular photobiomodulation on motor deficits and brain perfusion images in intractable myasthenia gravis: A case report

BACKGROUND Myasthenia gravis(MG)is an autoimmune disorder caused by neuromuscular junction failure characterized by muscle weakness and fatigability.We herein report a case of MG that received intravascular laser irradiation of blood(ILIB)interventions and regained muscle power and better quality of life.To our knowledge,no previous study has investigated the benefits of ILIB treatment on patients with MG.We also evaluated the changes in brain perfusion scan and the MG activities of daily living(MG-ADL)and quantitative MG(QMG)scales.CASE SUMMARY A 59-year-old man presented to our outpatient hospital experiencing ptosis,diplopia,fibromyalgia,muscle fatigue,and fluctuating weakness in his limbs for 1 year.Based on his history,physical examination,and laboratory investigations,the final diagnosis was a flare-up of MG with poor endurance and muscle fatigue.The patient agreed to receive ILIB.Brain single-photon emission computed tomography(SPECT)was performed both before and after ILIB therapy.After receiving three courses of ILIB,the brain SPECT images showed greatly increased perfusion of the frontal lobe and anterior cingulate gyri.The patient’s MG-ADL scale score decreased markedly from 17/24 to 3/24.The QMG scale score also decreased remarkably from 32/39 to 9/39.The symptoms of MG became barely detectable and the patient was able to perform his activities of daily living and regain muscle power.CONCLUSION ILIB might have beneficial effects on MG,and brain SPECT images provided direct evidence of a positive correlation between ILIB and clinical performance.

Clinical manifestation and humoral immuno-function of myasthenia gravis patients with abnormal and normal thymus gland

BACKGROUND： Myasthenia gravis （MG） is an autoimmune disease which mainly affects neuromuscular junctions. The ages, modified Osserman classification and clinical manifestation and humoral immunol function of MG with and without thymic abnormality are different. OBJECTIVE： To explore the clinical manifestation and humoral immuno-function of MG with abnormal and normal thymus gland. DESIGN ： Contrast observation SETTTNG ： Department of Neurology, the Third Affiliated Hospital of Sun Yat-sen University PARTZCZPANTS ： A total of 49 inpatients with MG were selected from the Third Affiliated Hospital of Sun Yat-sen University from March 2000 to August 2005. All the patients had typical clinical manifestation of MG and positive neostigmine test. All the patients knew and agreed the laboratory examinations. There were 22 males and 27 females of 2-69 years old. Chest MRI or CT scan were performed to reveal thymus gland abnormality. According to whether there was tumor in superior mediastinum, all patients were divided into 2 groups, abnormal and normal groups. Normal thymus gland group （n=30） contained 16 males and 14 females of 6-43 years old. Abnormal thymus gland group （n=19） contained 6 male and 13 female of 2-69 years old, METHODS： ① All patients were questioned about initial symptoms. Meanwhile, main clinical manifestations were recorded at hospital admission. ② 7180A automatic biochemical analyzer and automatic microplate reader were used in detecting seroimmunity index. The levels of C3, C4, IgG, IgA, IgM and CH50 in blood serum were analyzed by nephelometry. ③ Clinical classification is based on modified Osserman classification. The patients with MG were divided into six types： I （Ocular myasthenia）, Ⅱ a （Mild generalized myasthenia）, Ⅱb （Moderately severe generalized myasthenia）, Ⅲ （Acute fulminating myasthenia）, Ⅳ（Late se- vere myasthenia）. MAZN OUTCOME MEASURES： ① Differences of initial symptoms and clinical manifestation of two group patients. ② Differences of age of onset and modified Osserman classification of two groups. ③The humoral immuno-functions of two groups were compared. RESULTS： All the 49 patients were involved in the final analysis of results. ① Differences of initial symptoms： Ptosis was the most common initial symptoms in both groups. Patients with ptosis of normal thymus gland were 25 （83%, 25/30）. Patients with ptosis of abnormal thymus gland were 13 （68%, 13/19）. Patients with normal thymus gland： dysphagia 2 （7%, 2/30）, diplopia 4 （13%, 4/30）, fatigue 4 （13%, 4/30）, dysarthria 3, （10 %, 13/30）. Patients with abnormal thymus gland： dysphagia 3 （16%, 3/19）, diplopia 6 （32%, 6/19）, fatigue 3 （16%, 3/19）, dysarthria 2 （10%, 2/19）. ② Differences of clinical manifestation of two groups： Ptosis was the most common clinical manifestation in both groups. Patients with ptosis of normal thymus gland were 29 （97%, 29/30）. Patients with ptosis of abnormal thymus gland were 15 （79%, 15/19）. The rates of fatigue and breathing disorder in patients with abnormal thymus gland were higher than patients with normal thymus gland. Myasthenia crisis occurred in 3 patients （16 %, 3/19） in abnormal thymus gland group, with 1 （3%, 1/30） in abnormal thymus gland group. ③ Differences of age of onset and modified Osserman classification： The rate of type ｜ （63%, 19/30） in patients with normal thymus gland was higher than patients （42%, 8/19） with abnormal thymus gland. The rates of type Ⅱ a, Ⅱ b and Ⅲ （58 %） in patients with abnormal thymus gland were higher than patients （37%, 8/19） with normal thymus gland. But no differences were found between two groups （P 〉 0.05）. Patient number of onset from 20 to 29 year old in abnormal group （47%） was higher than that in normal group （20%）. Comparison of two groups was X2=4.10 and P 〈 0.05.④ Comparison of the humoral immunol indexes of two groups： The levels of IgG, IgA, C3 and CH50 in abnormal group were higher than those in normal group. But no differences were found between two groups （P 〉 0.05）. CONCLUSZON： ① Ptosis was the most common initial symptom and clinical feature in both groups. ② Clinical manifestation in abnormal group were more severe, and ages of onset in abnormal group were more young.③ The humoral immuno indexes of two groups were not significantly different.

Successful Combination Therapy of Nivolumab and Radiation for Massive Mucosal Melanoma in Patient with Autoimmune-Myasthenia Gravis, Systemic Lupus Erythematosus and Rheumatoid Arthritis

A 58-year-old woman complicated with autoimmune diseases of myasthenia gravis (MG), rheumatoid arthritis, and systemic lupus erythematosus noticed an irregular black macule on her bilateral major labia, which was diagnosed as malignant melanoma. The melanoma lesion involving the vagina, uterus, and ventral side of rectum was not operable and was treated with nivolumab and concurrent radiotherapy with good control of the MG. This resulted in remarkable tumor shrinkage, possibly due to synergistic effects of both treatments. To our knowledge, few reports have described the effectiveness of combination therapy with nivolumab and radiation for malignant melanoma. The present case showed an enhanced anti-tumor effect with combination therapy.

Effectiveness of progressive resistance training in improving muscle strength in myasthenia gravis:a single subject study design

Background:To investigate the effectiveness of the progressive resistance training(PRT)using thera band in improving muscle strength in myasthenia gravis(MG).Methods:In this prospective study,12 MG patients with generalised stable disease performed progressive resistance training with thera band for four times per week for 24 weeks.The isometric muscle force of shoulder abductors,biceps brachii and knee extensors using handheld dynamometer,Myasthenia Gravis Composite(MGC)score,Quantitative myasthenia gravis score(QMG),were assessed before and after the training period.Results:Progressive resistance training was well tolerated,and the isometric muscle strength was significantly improved in shoulder abductors,biceps brachii and knee extensors(P<0.05).The disease course(QMG and MGC)was slowed down and improved(P<0.05).Conclusion:Progressive resistance training is effective in improving muscle strength specifically in most affected muscles in MG.

Histologic Classification of Thymoma and Its Relationship with Myasthenia Gravis and Clinical Stages of the Tumor

Objective： To investigate the relationship among the latest WHO classification of thymoma, myasthenia gravis （MG） and clinical stages. Methods： To review the pathological sections of 74 patients with thymoma from 1980-2004 using WHO classification （1999）, the statistical software was used to analyze the relationship among the WHO classification, MG and clinical stages. Results： （1） Two cases of type A, 23 cases of type AB, 4 cases of type B1, 27 cases of type B2, 16 cases of type B3 and 2 cases of type C were classified. Type B2 more likely accompanied MG （P〈0.05）, while none with MG occurred for type C. （2） One patient was in stage Ⅰ, 30 were in stage Ⅱ, 38 were in stage Ⅲ, and 5 were in stage Ⅳ. The latest histologic classification was significantly correlated with Masaoka stages （P〈0.01）. Conclusion： The latest WHO classification was correlated with occurrence of MG and finely reflected clinical stage. It can also evaluate the prognosis of patients.

Surgical approaches for stage Ⅰ and Ⅱ thymoma-associated myasthenia gravis:feasibility of complete video-assisted thoracoscopic surgery (VATS) thymectomy in comparison with trans-sternal resection

Complete resection could be achieved in virtually all myasthenic patients with Masaoka stage I and II thymoma us- ing the trans-sternal technique. Whether this is appropriate for minimally invasive approach is not yet clear. We evalu- ated the feasibility of complete video-assisted thoracoscopic surgery （VATS） thymectomy for the treatment of Ma- saoka stage I and Ⅱ thymoma-associated myasthenia gravis, compared to conventional trans-sternal thymectomy. We summarized 33 patients with Masaoka stage I and II thymoma-associated myasthenia gravis between April 2006 and September 2011. Of these, 15 patients underwent right-sided complete VATS （the VATS group） by us- ing adjuvant pneuomomediastinum, comparing with 18 patients using the trans-sternal approach （the T3b group）. No intraoperative death was found and no VATS case required conversion to median sternotomy. Significant differences between the two groups regarding duration of surgery and volume of intraoperative blood loss （P = 0.001 and P 〈 0.001, respectively） were observed. Postoperative morbidities were 26.7% and 33.3% for the VATS and T3b groups, respectively. All 33 patients were followed up for 12 to 61 months in the study. The cumulative probabilities of reaching complete stable remission and effective rate were 26.7% （4/15） and 93.3% （14/15） in the VATS group, which had a significantly higher complete stable remission and effective rate than those in the T3b group （P = 0.026 and P = 0.000, respectively）. We conclude that VATS thymectomy utilizing adjuvant pneuomo- mediastinum for the treatment of stage I and II thymoma-associated myasthenia gravis is technically feasible but deserves further investigation in a large series with long-term follow-up.

Myasthenia gravis and thymic neoplasms: A brief review

Thymoma is the most common mediastinal tumor. They have varied presentation ranging from asymptomatic incidental mediastinal masses to locally extensive tumor with compressive symptoms and distant metastases. They have frequent association with various paraneoplastic syndromes(PNS). The most common PNS associated with thymoma is myasthenia gravis(MG). Patients of thymoma with MG have a favourable outcome due to early disclosure of the disease. Histologically they are classified into five subtypes and MasaokaKoga staging system is used for staging. Surgery, chemotherapy and radiotherapy play an important role along with anti-myasthenia drugs. This review would like to highlight the association of thymoma with MG and associated clinical and therapeutic issues.

TUMOR NECROSIS FACTOR-ALPHA POLYMORPHISM AND SECRETION IN MYASTHENIA GRAVIS

Objective To analyze the relationship between tumor necrosis factor-alpha (TNFα) gene promoter -308 polymorphism and myasthenia gravis (MG) in Chinese and analyze secretion of TNFα in peripheral blood mononuclear cells (PBMC) in MG patients. Methods A biallelic polymorphism at position -308 in the promoter of TNFα gene was screened by PCR amplification and NcoI recognition site. One hundred and twenty-three MG cases and 115 healthy controls were included in this study. MG patients were classified to different groups according to clinical type, age at onset, and sex respectively. PBMC were isolated from 20 patients and 20 healthy controls, and then cultured in the presence or absence of phytohemag- glutinin (PHA) and acetycholine receptors (AchR). The supernatants were harvested after incubation and stored until TNFα was assayed by enzyme-linked immunosorbent assay. Results The frequency of TNFα-308 allele 2 (A) was found significantly increase in MG patients and showed a trend especially in late onset (≥ 40 years) and male patients (P < 0.05). The allele A had no relationship with thymic pathogenesis in MG patients. But frequency of allele A was significantly higher in general type than in ocular type (P < 0.05). MG patients had a higher inducible level of TNFα by PHA and AchR, and could be down regulated after treatment. Conclusion Polymorphism in TNFα gene promoter -308 is associated with onset of MG. The microsatellite allele TNFα2 confer risk for the development of MG in Chinese patients. MG patients have a higher inducible level of TNFα.

Effectiveness of Thymectomy in Non-thymomatous Myasthenia Gravis: a Systematic Review

There is continuous debate regarding the effectiveness of thymectomy in the treatment of non-thymomatous myasthenia gravis （MG）. This systematic review was undertaken to determine whether thymectomy was effective in non-thymomatous MG. We retrieved articles published between January 1980 and September 2013. Sixteen cohort studies were included. Given the considerable het- erogeneity, we used a descriptive method instead of statistical synthesis. The median relative rates （RRs） and their interquartile ranges were used to estimate the magnitude of benefit. Compared to conserva- tively treated MG patients, thymectomized patients had higher survival, clinical remission, pharma- cologic remission and improvement rates, and RRs were 1.07 （1.01-1.17）, 1.83 （0.82-2.99）, 1.55 （1.22-1.95） and 1 （1.00-1.09）, respectively. Subgroup analyses showed that patients with moderate to severe generalized MG benefited more from thymeetomy, with RRs of survival and pharmacologic re- mission increasing to 1.35 （1.24-1.49） and 2.68 （1.73-4.17）, respectively. These results suggested that thymectomy might be an effective procedure in non-thymomatous MG patients. The patients with mod- erate to severe generalized MG might benefit more. Taking into account the poor methodological qual- ity of present studies, more well-designed prospective randomized controlled trials （RCTs） are still re- quired to reach uneouivocal conclusion.

Investigation on the Mechanism of Exacerbation of Myasthenia Gravis by Aminoglycoside Antibiotics in Mouse Model

To investigate the underlying mechanism of the exacerbation of myasthenia gravis by aminoglycoside antibiotics. C57/BL6 mice were immunized with acetylcholine receptor (AChR), extracted from electric organ of Narcine timilei according to Xu Haopeng's methods, in complete Fruend's adjuvant (CFA) to establish experimental autoimmune myasthenia gravis (EAMG). EAMG mice were divided randomly into 5 groups: MG group, NS group and three antibiotics groups. The clinical symptom scores of mice were evaluated on d7 after the last immunization and d14 of antibiotics treatment. Repetitive nerve stimulation (RNS) was performed and the levels of anti-AChR antibody (AChR-Ab) were tested at the same time. The mean clinical symptom grades of gentamycin group (1.312, 2.067), amikacin group (1.111, 1.889) and etimicin group (1.263, 1.632) were significantly higher than those of MG group (1.000, 1.200) (P<0.05). The positive rates of RNS of three antibiotics groups were 69.23 %, 58.82 % and 63.16 % respectively, which were significantly higher than those of MG group and NS group (40.00 %, 40.00 %, P<0.05). The AChR-Ab level in serum and the expression of AChR on neuromuscular junction (NMJ) of mice in three antibiotics groups were also higher than those of MG group. Our results indicated that aminoglycoside antibiotics could aggravate the symptom of myasthenia gravis. The exacerbation of myasthenia gravis by these antibiotics probably involves competitively restraining the release of acetylcholine from presynaptic membrane, impairing the depolarization of postsynaptic membrane, depressing the irritability of myocyte membrane around the end-plate membrane and consequently leading to the blockade of neuromuscular junction.

Myasthenia gravis exacerbation and diarrhea associated with erythromycin treatment

An important problem in management of the case with myasthenia gravis (MG) is the control of exacerbation. There are several possible causes of exacerbation of MG including the use of drug. Here, the authors report a case of MG exacerbation and diarrhea associated with erythromycin treatment.

Reduction of natural regulatory T cells in thymomas accompanying myasthenia gravis and its possible association with Foxp3 and thymic stromal lymphopoietin

Objective: To investigate the expression of both thymic regulatory T cells (CD4+CD25+Foxp3+cells, Treg) and thymic stromal lymphopoietin (TSLP) in thymomas accompanying myasthenia gravis. Methods: We used immunohistochemistry and real-time reverse trancription polymerase chain reaction (real-time RT-PCR) techniques to determine Foxp3+ Treg counts and the expression levels of Foxp3 mRNA and TSLP mRNA in thymomas of 23 MG patients and thymuses of 4 healthy controls. Results: The CD4+ Foxp3+ nTreg (natural regulatory T cells) counts in thymomas were significantly lower than those in normal thymuses (P<0.01), and the expression levels of Foxp3 mRNA and TSLP mRNA were also lower in thymomas(P<0.01). Among the thymoma types, type B1 thymoma had the highest Foxp3+ nTreg count and standard values of Foxp3 mRNA and TSLP mRNA. There was a strong positive correlation between the mRNA transcriptional levels of Foxp3 and TSLP. Conclusion: The insufficient expression of Foxp3 in thymoma, which may be caused by decreased transcription of TSLP, may result in the reduction of Tregs and cause autoimmune disorders.

FcRn inhibitors:a novel option for the treatment of myasthenia gravis

Myasthenia gravis is an acquired,humoral immunity-mediated autoimmune disease characterized by the production of autoantibodies that impair synaptic transmission at the neuromuscular junction.The intervention-mediated clearance of immunoglobulin G(IgG)was shown to be effective in controlling the progression of the disease.The neonatal Fc receptor(FcRn)plays a key role in prolonging the serum half-life of IgG.Antagonizing FcRn to prevent its binding to IgG can accelerate the catabolism of the latter,resulting in decreased levels of IgG,including pathogenic autoantibodies,thereby achieving a therapeutic effect.In this review,we detail the substantial research progress,both basic and clinical,relating to the use of FcRn inhibitors in the treatment of myasthenia gravis.