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

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Neural Regeneration Research 2023年第8期18卷 1637-1644页

作者： Li-Na Zhu Hai-Man Hou Sai Wang Shuang Zhang Ge-Ge Wang Zi-Yan Guo Jun Wu Department of Neurology The First Affiliated Hospital of Zhengzhou UniversityZhengzhouHenan ProvinceChina

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.

关键词： batoclimab clinical trial efgartigimod FcRn FcRn inhibitors immunoglobulin G(IgG) myasthenia gravis nipocalimab rozanolixizumab

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

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World Journal of Clinical Cases 2022年第24期10卷 8718-8727页

作者： Chiao-Hsin Lan Yu-Che Wu Cheng-Chun Chiang Shin-Tsu Chang School of Medicine National Defense Medical CenterTaipei 114201Taiwan Department of Medical Education and Research Kaohsiung Veterans General HospitalKaohsiung 813414Taiwan Department of Physical Medicine and Rehabilitation Kaohsiung Veterans General HospitalKaohsiung 813414Taiwan Department of Physical Medicine and Rehabilitation Tri-Service General HospitalTaipei 114202Taiwan

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.

关键词： myasthenia gravis Intravascular laser irradiation of blood myasthenia gravisactivities of daily living scale Quantitative myasthenia gravis scale Single-photon emission computed tomography Case report

Intraoperative cardiogenic shock induced by refractory coronary artery spasm in a patient with myasthenia gravis: A case report

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World Journal of Clinical Cases 2023年第36期11卷 8589-8594页

作者： Cheng-Wei Hsu Chuen-Chau Chang Chao-Shun Lin Department of Anesthesiology Taipei Medical University HospitalTaipei 110Taiwan Department of Anesthesiology School of MedicineCollege of MedicineTaipei Medical UniversityTaipei 110Taiwan

BACKGROUND Coronary artery spasm(CAS)is a rare but critical condition during surgery.Clinical manifestations can vary from only subtle electrocardiography change to sudden death.In this case report,we present the case of a patient with myasthenia gravis(MG)who developed refractory CAS-related cardiogenic shock during thymoma surgery.CASE SUMMARY A 61-year-old man had a history of cigarette smoking and coronary artery disease with a bare metal stent placed.Three months ago,he suffered from coronary spasms,with three vessels involved,after surgery for cervical spine injury.He started having progressive dysphagia 4 wk prior and was diagnosed with MG via serologic tests,and computed tomography declared a thymoma in the anterior mediastinum.After the symptoms of MG subsided,he was referred for thy-mectomy.The operation was uneventful until the closing of the sternal wound.Electrocardiography showed sudden onset ST elevation,followed by ventricular tachycardia and severe hypotension.Cardiopulmonary cerebral resuscitation was initiated immediately with electrical defibrillation,extracorporeal membrane oxygenation was performed due to refractory cardiogenic shock,and the patient was transferred to an angiography room.Angiography showed diffuse CAS with three vessels involved.Intracoronary isosorbide dinitrate and adenosine were administered,and then the patient was transferred to the intensive care unit.CONCLUSION Our case highlights the importance of being prepared for clinical situations such as the one described here and suggests the necessity of developing an appropriate anesthesia plan that includes proactive analgesia and preemptive coronary vaso-dilators.

关键词： Coronary spasm myasthenia gravis Thymectomy Shock Case report

myasthenia gravis in pediatric and elderly patients

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Chinese Medical Journal 2003年第10期116卷 1578-1581页

作者：刘伟刘国津范志民盖学良 Department of Thoracic Surgery and Bethune-Laval Oncology Unit (BLOU) the First HospitalJilin University

Objective To determine whether the clinical and pathologic characteristics and prognoses of myasthenia gravis (MG) patients below 15 years differ from those patients over 50 years after thymectomy. Methods We reviewed the registry material of 30 pediatric and 32 elderly MG patients after thymectomy,including their age,sex,clinical classification,pathological types,and prognoses. The Chi-square test or Wilcoxon’s rank-sum test was used to determine the statistical differences between the children and elderly groups.Results No significant difference was seen in sex distribution between the two groups (Chi-square test, P =0.625),but there were differences in clinical classification: more type Ⅰ was observed in the pediatric group than in the elderly group,but more type Ⅱor Ⅲ was seen in the elderly group (Wilcoxon's rank-sum test,P <0.001). As to pathological types,the pediatric group was also significantly different from the elderly group (Chi-square test,P <0.01). All of the patients (100%) in the pediatric group had thymus hyperplasia,but in the elderly group more than half (56.26%) were found to have thymoma (benign or malignant). The prognoses after thymectomy were better in the pediatric group than in the elderly group (Wilcoxon's rank-sum test,P <0.001). Conclusions Because the prognoses are generally better than those of the elderly patients,we should be careful when operating on pediatric patients of ocular type. The elderly patients tend to receive more aggressive treatment because of more severe generalized types often associated with thymoma and poor prognoses. Both pediatric and elderly patients are seldom associated with other autoimmune disease.

关键词： myasthenia gravis age thymectomy prognosis

Clinical significance of detection of antibodies to fetal and adult acetylcholine receptors in myasthenia gravis

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Neuroscience Bulletin 2012年第5期28卷 469-474页

作者： Qi-Guang Shi Zhi-Hong Wang Xiao-Wei Ma Da-Qi Zhang Chun-Sheng Yang Fu-Dong Shi Li Yang Department of Neurology and Tianjin Neurological Institute Tianjin Medical University General Hospital Tianjin 300052 China Department of Neurology Barrow Neurological Institute St. Joseph's Hospital and Medical Center Phoenix Arizona 85013 USA

Objective To evaluate the frequency, distribution and clinical significance of the antibodies to the fetal and/or adult acetylcholine receptor （AChR） in patients with myasthenia gravis （MG）. Methods AChR antibodies were detected by cell-based assay in the serum of ocular MG （OMG）（n = 90） and generalized MG （GMG） patients （n = 110）. The fetal-type （2α： β： γ： δ） and adult-type （2α： β： ε： δ） AChR were used as antigens, and their relevance to disease presentation was assessed. Results The overall frequencies of anti-adult and anti-fetal AChR antibodies were similar in all 200 patients examined, with 14 having serum specific to the AChR-γ subunit, and 22 to the AChR-ε subunit. The overall sensitivity when using the fetal and adult AChR antibodies was higher than that when using the fetal AChR antibody only （P = 0.015）. Compared with OMG patients, the mean age at disease onset and the positive ratio of antibodies to both isoforms of the AChR were significantly higher in patients who subsequently progressed to GMG. Older patients and patients with both anti-fetal and anti-adult AChR antibodies had a greater risk for developing generalized disease [odds ratio （OR）, 1.03; 95% confidence interval （CI）, 1.01–1.06 and OR, 5.09; 95% CI, 2.23–11.62]. Conclusion Using both fetal-and adult-type AChRs as the antigens may be more sensitive than using either subtype. Patients with serum specific to both isoforms are at a greater risk of progressing to GMG. Patients with disease onset at an advanced age appear to have a higher frequency of GMG conversion.

关键词： myasthenia gravis acetylcholine receptor antibodies acetylcholine receptor subunit cell-based assay adult fetal

Fusion protein of single-chain variable domain fragments for treatment of myasthenia gravis

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Neural Regeneration Research 2014年第8期9卷 851-856页

作者： Fangfang Li Fanping Meng Quanxin Jin Changyuan Sun Yingxin Li Honghua Li Songzhu Jin Department of Immunology and Pathogenic Biology College of MedicineYanbian University

Single-chain variable domain fragment （scFv） 637 is an antigen-specific scFv of myasthenia gravis. In this study, scFv and human serum albumin genes were conjugated and the fusion pro-tein was expressed in Pichia pastoris. The afifnity of scFv-human serum albumin fusion protein to bind to acetylcholine receptor at the neuromuscular junction of human intercostal muscles was detected by immunolfuorescence staining. The ability of the fusion protein to block myas-thenia gravis patient sera binding to acetylcholine receptors and its stability in healthy serum were measured by competitive ELISA. The results showed that the inhibition rate was 2.0-77.4%, and the stability of fusion protein in static healthy sera was about 3 days. This approach suggests the scFv-human serum albumin is a potential candidate for speciifc immunosuppressive therapy of myasthenia gravis.

关键词： nerve regeneration myasthenia gravis acetylcholine receptor anti-acetylcholine re-ceptor antibody single-chain variable domain fragment human serum albumin fusion protein immunosuppressive therapy autoimmune disease NSFC grant neural regeneration

Prognosis of the short-term efficacy of thymectomy for myasthenia gravis with serum protein profiles

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Chinese Medical Journal 2009年第14期122卷 1625-1630页

作者： WU Guo-yong PANG Jing-zhuo CHENG Chao LU Jian-jun MA Jun GU Yong ZHONG Fuo-tian LUO Hong-he Thoracic Surgery Department First Affiliated Hospital of Sun Yat-Sen University Guangzhou Guangdong 510080 China

Background The extended thymectomy for myasthenia gravis （MG） is currently available, but in 20%-40% of the patients the results were not satisfactory. There are no ideal indicators forecasting surgical results before operation. The surface enhanced laser desorption ionization-time of flight-mass spectroscopy （SELDI-TOF-MS） is a currently new technique for detection of protein profiles, and some progresses have been made in cancer diagnosis and efficacy evaluation, but there is no report on efficacy forecasting of MG surgery. This study aimed to establish an efficacy prognosis model for forecasting the efficacy of surgery for MG by analysis of serum protein profiles of MG patients before surgery. Methods Fifty-six MG patients 6 months after extended thymectomy were enrolled in the study. They were classified into effective or non-effective groups according to symptoms and medication. Their pre-operative blood samples were analyzed for protein profiles by the SELDI-TOF MS technique, and protein peaks were identified for establishment of the efficacy prognosis model of MG surgery. Additional 100 MG patients were subjected to model validation and their pre-operation protein profiles reviewed for post-operative results. The results were compared with those of the post-operative follow-up so as to validate the prognosis model. Results For the model establishment, symptoms were improved in 33 patients and not improved in 18 patients, with an effective rate of 64.7%. Five （8.9%） patients were lost to follow-up. Within the molecular weight range of 1 000 to 20 000, 3 specific protein peaks were found to be significantly different between the effective and non-effective groups, ie M4110-76, M3394-58, and M1258-55. Using the efficacy prognosis model constructed with these data, the accuracy rate of classification was 87.9% for the effective group, and 83.3% for the non-effective group, with a total accuracy rate of 86.3%. For the model evaluation, 2 （8.9%） patients were lost to follow-up, 62 patients were effective and 36

关键词： enhanced laser desorption ionization-time flight-mass spectroscopy serum protein profiles myasthenia gravis surgery efficacy

Perioperative and long-term outcome of thymectomy for myasthenia gravis： comparison of surgical approaches and prognostic analysis

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Chinese Medical Journal 2013年第1期126卷 34-40页

作者： LIU Cheng-wu LUO Meng MEI Jian-dong ZHU Yun-ke PU Qiang MA Lin CHE Guo-wei LIN Yi-dan WU Zhu WANG Yun KOU Ying-li LIU Lun-xu Department of Thoracic Surgery West China Hospital SichuanUniversity Chengdu Sichuan 610041 China Department of Thoracic Surgery Guizhou Provincial People'sHospital Guiyang Guizhou 550002 China

Background Thymectomy is an established treatment for myasthenia gravis （MG）, and video-assisted thoracoscopic surgery （VATS） thymectomy has become an acceptable surgical procedure. This study aimed to compare the results of VATS thymectomy and open thymectomy and to identify the prognostic factors after thymectomy. Methods The clinical data of 187 consecutive thymectomies performed between July 2000 and December 2009 were retrospectively reviewed; 75 open thymectomies and 112 VATS thymectomies. Clinical efficacy and variables influencing outcome were assessed by Kaplan-Meier survival curves and Cox proportional hazards regression analysis. Results The operative blood loss in the VATS group was significantly less than that in the open group （（62.14＋55.43） ml vs. （137.87＋165.25） ml, P 〈0.05）. The postoperative crisis rate increased with the severity of preoperative MG and the prescription dose of anticholinesterase. Complete follow-up information of patients more than 12 months after the thymectomy was obtained on 151 cases, 89 cases from the VATS group and 62 cases from the open group, with a mean follow-up period of 59.3 months, range from 12 to 117 months. Complete stable remission （CSR） was the end point for evaluation of the treatment results. The overall five-year CSR rate was 57.5%. Two good prognostic factors were identified; preoperative prescription of anticholinesterase alone （P=0.035） and non-thymomatous MG （P=0.003）. The five-year CSR rate of the ocular type of MG reached a high level of 67.4%. Conclusions Thymectomy can achieve good long-term CSR in MG, and VATS is an ideal alternative method. High-dose prescription of anticholinesterase and the advanced stage by myasthenia gravis Foundation of America （MGFA） classification have higher risks of postoperative crisis. Preoperative prescription of anticholinesterase alone and non-thymomatous MG are good prognostic factors. Thymectomy should also be considered for the ocular tvioe of MG.

关键词： myasthenia gravis thymectomy video-assisted thoracoscopic surgery transsternal thymectomy prognosis

Effect of treatment with Fufang Huangqi decoction(复方黄杞汤剂) on dose reductions and discontinuation of pyridostigmine bromide tablets, prednisone, and tacrolimus in patients with type Ⅰ or Ⅱ myasthenia gravis

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Journal of Traditional Chinese Medicine 2022年第5期42卷 810-817页

作者： SHAN Caifeng ZHANG Jingsheng LIN Yi HUANG Xueshi WANG Zhanyou PAN Haiou QIAO Wenjun Affiliated Hospital of Liaoning University of Traditional Chinese Medicine Liaoning Provincial Key Laboratory for Diagnosis and Treatment of Myasthenia GravisLiaoning University of Traditional Chinese MedicineShenyang 110033China Department of General Surgery the First People's Hospital of ShenyangShenyang 110091China Institute of Microbial Pharmaceuticals College of Life and Health SciencesNortheastern UniversityShenyang 110819China Institute of Health Sciences Key Laboratory of Medical Cell Biology of Ministry of EducationChina Medical UniversityShenyang 110122China Liaoning University of Traditional Chinese Medicine foreign language collegeLiaoning University of Traditional Chinese MedicineShenyang 110033China

OBJECTIVE: To investigate the clinical efficacy of Fufang Huangqi decoction(复方黄杞汤剂) in combination with pyridostigmine bromide tablets, prednisone, and tacrolimus in the treatment of type Ⅰ and Ⅱ myasthenia gravis(MG) through changes in the clinical symptom scores of 100 patients with type Ⅰ and Ⅱ MG. This study also aimed to examine dose reductions and discontinuation of these 3 Western medicines after administration of Fufang Huangqi decoction. METHODS: The clinical data on 100 patients with type I or II MG who were treated in the outpatient department of the Affiliated Hospital of Liaoning University of Traditional Chinese Medicine, China, between June 2017 and June 2020 were collected. The patients were divided into 4 groups based on whether they had taken pyridostigmine bromide tablets, prednisone, and/or tacrolimus at the time of their hospital visit: the Fufang Huangqi decoction group(group A), the pyridostigmine bromide tablets + Fufang Huangqi decoction group(group B), the pyridostigmine bromide tablets + prednisone + Fufang Huangqi decoction group(group C), and the pyridostigmine bromide tablets + tacrolimus + Fufang Huangqi decoction group(group D). The average treatment time was(15.6 ± 11.5) months(range: 0.5-55 months). Changes in the clinical symptom scores of the 4 groups of patients after medication administration and dose reductions and discontinuation of the 3 Western medicines were analyzed. RESULTS: An overall effectiveness rate of 86.00% was achieved in the 100 patients after treatment for(15.6 ± 11.5) months(range 0.5-55 months). The effectiveness rates were 85.71% in group A, 88.24% in group B, 76.92% in group C, and 80.00% in group D. The dosage of pyridostigmine bromide was reduced for 69.12% of the patients in group B for the first time after(4.2 ± 4.1) months, and 45.59% of the patients in group B discontinued pyridostigmine bromide after(8.8 ± 6.1) months. The dosage of pyridostigmine bromide was reduced for 46.15% of the patients in group C for the first time after(5.3 ± 3.4) months,

关键词： myasthenia gravis drug tapering drug-related side effects and adverse reactions Fufang Huangqi decoction