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Article: Effectiveness of early glucocorticoids in myasthenia gravis: a retrospective cohort study.

Zhen, Lulu / Zhao, Xue / Li, Wenbo / Wu, Jinru / Shang, Haodong / Chen, Shufan / Zhu, Xiaoyan / Wang, Yiren / Yu, Xiaoxiao / Hu, Guanlian / Sun, Zhan / Zhang, Yingna / Zhang, Jing / Fang, Hua / Zhang, Yunke / Zhang, Qingyong / Cui, Xinzheng / Lv, Jie / Yang, Junhong /

Gao, Feng

Frontiers in neurology

2023 Volume 14, Page(s) 1259484

Abstract: Purpose: This study aimed to clarify the effect of early glucocorticoid (GC) application on achieving minimal manifestation (MM) status or better in the treatment of myasthenia gravis (MG) in the early clinical phase.: Methods: A retrospective ... ...

Abstract	Purpose: This study aimed to clarify the effect of early glucocorticoid (GC) application on achieving minimal manifestation (MM) status or better in the treatment of myasthenia gravis (MG) in the early clinical phase. Methods: A retrospective analysis was performed using data from 336 patients with MG who received GC therapy from January 2015 to September 2022 in the Zhengzhou University Henan Institute of Medical and Pharmaceutical Sciences Myasthenia Gravis Biobank (ZMB). Patients were divided into two groups: the early mono-GC group (treated with GC within 6 months of MG onset) and the delayed mono-GC group. Results: Kaplan-Meier analysis showed that the early mono-GC group achieved MM status earlier and more frequently than the delayed mono-GC group (log-rank test, Conclusion: Early intervention with GC led to better long-term outcomes and reduced the necessary maintenance dose of oral GC for patients with MG. EOMG and OMG were positive predictors of MM status or better with mono-GC.
Language	English
Publishing date	2023-12-19
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2564214-5
ISSN	1664-2295
ISSN	1664-2295
DOI	10.3389/fneur.2023.1259484
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Article ; Online: Clinical characteristics of anti-AChR-MuSK-LRP4 antibody-negative myasthenia gravis in China.

Li, Wenbo / Liu, Peipei / Cui, Weike / Wang, Shumin / Ji, Ying / Zhang, Linyuan / He, Xiaoxiao / Zhou, Shuxian / Shen, Tong / Zhao, Xue / Lv, Jie / Zhang, Yingna / Zhang, Jing / Fang, Hua / Yang, Junhong / Zhang, Yunke / Cui, Xinzheng / Zhang, Qingyong / Gao, Feng

Muscle & nerve

2023 Volume 67, Issue 6, Page(s) 481–488

Abstract: Introduction/aims: Descriptions of the clinical characteristics of anti-AChR-MuSK-LRP4 antibody-negative myasthenia gravis (triple-negative myasthenia gravis, TNMG) are lacking in the current literature. Therefore, we investigated the clinical ... ...

Abstract	Introduction/aims: Descriptions of the clinical characteristics of anti-AChR-MuSK-LRP4 antibody-negative myasthenia gravis (triple-negative myasthenia gravis, TNMG) are lacking in the current literature. Therefore, we investigated the clinical characteristics of TNMG in Chinese patients. Methods: We retrospectively analyzed 925 patients with MG registered in the Department of Neuroimmunology, Henan Institute of Medical and Pharmaceutical Sciences from January 2015 to March 2021. Results: One hundred six patients diagnosed with TNMG were included in the study. The average age of onset was 32.4 y, with a male-to-female ratio of 1:1. The age of onset showed a bimodal distribution: 0-9 y and 40-49 y. Adult patients were more likely to have weakness of limb and bulbar muscles (p < .05). Thymic hyperplasia was found in 20.2% of the patients. Younger patients were more likely to relapse. The rate of adult early-onset myasthenia gravis reaching complete stable remission and pharmacological remission was 47.6%, and the prognosis was better than that in juvenile-onset myasthenia gravis (p = .019). Older age of onset was the only risk factor for the development of generalized TNMG from ocular TNMG (R = 1.046, p = .002, 95% confidence interval 1.017-1.077). Discussion: This study showed that the clinical characteristics of patients with TNMG varied among the different age groups. Significant findings included a bimodal distribution of onset age, coexisting thymic hyperplasia, and a generally favorable prognosis.
MeSH term(s)	Adult ; Humans ; Male ; Female ; Receptors, Cholinergic ; Retrospective Studies ; Thymus Hyperplasia ; Receptor Protein-Tyrosine Kinases ; Autoantibodies ; Neoplasm Recurrence, Local ; Myasthenia Gravis/diagnosis ; Myasthenia Gravis/epidemiology ; China/epidemiology ; LDL-Receptor Related Proteins
Chemical Substances	Receptors, Cholinergic ; Receptor Protein-Tyrosine Kinases (EC 2.7.10.1) ; Autoantibodies ; LRP4 protein, human ; LDL-Receptor Related Proteins
Language	English
Publishing date	2023-04-03
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	438353-9
ISSN	1097-4598 ; 0148-639X
ISSN (online)	1097-4598
ISSN	0148-639X
DOI	10.1002/mus.27822
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Article ; Online: Sorting nexin 17 increases low-density lipoprotein receptor-related protein 4 membrane expression: A novel mechanism of acetylcholine receptor aggregation in myasthenia gravis.

He, Xiaoxiao / Zhou, Shuxian / Ji, Ying / Zhang, Yingna / Lv, Jie / Quan, Shangkun / Zhang, Jing / Zhao, Xue / Cui, Weike / Li, Wenbo / Liu, Peipei / Zhang, Linyuan / Shen, Tong / Fang, Hua / Yang, Junhong / Zhang, Yunke / Cui, Xinzheng / Zhang, Qingyong / Gao, Feng

Frontiers in immunology

2022 Volume 13, Page(s) 916098

Abstract: Myasthenia gravis (MG) is characterized by autoimmune damage to the postsynaptic membrane of the neuromuscular junction (NMJ) with impaired postsynaptic acetylcholine receptor (AChR) aggregation. Low-density lipoprotein receptor-related protein 4 (LRP4) ... ...

Abstract	Myasthenia gravis (MG) is characterized by autoimmune damage to the postsynaptic membrane of the neuromuscular junction (NMJ) with impaired postsynaptic acetylcholine receptor (AChR) aggregation. Low-density lipoprotein receptor-related protein 4 (LRP4) plays an important role in AChR aggregation at endplate membranes
MeSH term(s)	Animals ; Mice ; Acetylcholine ; LDL-Receptor Related Proteins ; Lipoproteins, LDL ; Myasthenia Gravis, Autoimmune, Experimental ; Receptor Protein-Tyrosine Kinases ; Receptors, Cholinergic ; Sorting Nexins/genetics
Chemical Substances	Acetylcholine (N9YNS0M02X) ; LDL-Receptor Related Proteins ; Lipoproteins, LDL ; Receptor Protein-Tyrosine Kinases (EC 2.7.10.1) ; Receptors, Cholinergic ; Sorting Nexins ; Snx17 protein, mouse
Language	English
Publishing date	2022-10-12
Publishing country	Switzerland
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2606827-8
ISSN	1664-3224 ; 1664-3224
ISSN (online)	1664-3224
ISSN	1664-3224
DOI	10.3389/fimmu.2022.916098
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Article ; Online: Antibodies to Full-Length Agrin Protein in Chinese Patients With Myasthenia Gravis.

Wang, Shumin / Yang, Haonan / Guo, Rongjing / Wang, Lulu / Zhang, Yingna / Lv, Jie / Zhao, Xue / Zhang, Jing / Fang, Hua / Zhang, Qingyong / Zhang, Yunke / Yang, Junhong / Cui, Xinzheng / Gao, Peiyang / Chang, Ting / Gao, Feng

Frontiers in immunology

2021 Volume 12, Page(s) 753247

Abstract: This study aimed to establish a cell-based assay (CBA) for the detection of agrin antibodies (Agrin-Ab) to explore the clinical features of agrin antibody-positive Chinese patients with myasthenia gravis (Agrin-MG). We developed a CBA based on the human ... ...

Abstract	This study aimed to establish a cell-based assay (CBA) for the detection of agrin antibodies (Agrin-Ab) to explore the clinical features of agrin antibody-positive Chinese patients with myasthenia gravis (Agrin-MG). We developed a CBA based on the human full-length agrin protein expressed in HEK293T cells for the reliable and efficient detection of Agrin-Ab. Clinical data and serum samples were collected from 1948 MG patients in 26 provinces in China. The demographic and clinical features of Agrin-MG patients were compared with those of other MG patient subsets. Eighteen Agrin-MG cases were identified from 1948 MG patients. Nine patients were Agrin-Ab positive, and nine were AChR-Ab and Agrin-Ab double-positive (Agrin/AChR-MG). Eleven (61.11%) patients were males older than 40 years of age. The initial symptom in 13 (81.25%) cases was ocular weakness. Occasionally, the initial symptom was limb-girdle weakness (two cases) or bulbar muscle weakness (one case). Agrin-MG patients demonstrated slight improvement following treatment with either acetylcholinesterase inhibitor or prednisone; however, the combination of the two drugs could effectively relieve MG symptoms. In China, Agrin-MG demonstrated seropositivity rates of 0.92%. These patients were commonly middle-aged or elderly men. The patients usually presented weakness in the ocular, bulbar, and limb muscles, which may be combined with thymoma. These patients have more severe diseases, although the combination of pyridostigmine and prednisone was usually effective in relieving symptoms.
MeSH term(s)	Age of Onset ; Aged ; Agrin/chemistry ; Agrin/genetics ; Agrin/immunology ; Autoantibodies/blood ; Autoantigens/chemistry ; Autoantigens/genetics ; Autoantigens/immunology ; China/epidemiology ; Cholinesterase Inhibitors/therapeutic use ; Female ; Geography, Medical ; HEK293 Cells ; Humans ; Male ; Middle Aged ; Muscle Weakness/etiology ; Myasthenia Gravis/ethnology ; Myasthenia Gravis/etiology ; Myasthenia Gravis/immunology ; Prednisone/therapeutic use ; Recombinant Proteins/immunology ; Thymoma/complications ; Thymus Neoplasms/complications
Chemical Substances	Agrin ; Autoantibodies ; Autoantigens ; Cholinesterase Inhibitors ; Recombinant Proteins ; Prednisone (VB0R961HZT)
Language	English
Publishing date	2021-12-08
Publishing country	Switzerland
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2606827-8
ISSN	1664-3224 ; 1664-3224
ISSN (online)	1664-3224
ISSN	1664-3224
DOI	10.3389/fimmu.2021.753247
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Article ; Online: Peripheral blood hsa-circRNA5333-4: A novel biomarker for myasthenia gravis.

Lv, Jie / Ren, Lu / Han, Sensen / Zhang, Jing / Zhao, Xue / Zhang, Yingna / Fang, Hua / Zhang, Linyuan / Yang, Haonan / Wang, Shumin / Yang, Junhong / Cui, Xinzheng / Zhang, Qingyong / Zhang, Yunke / Gao, Feng

Clinical immunology (Orlando, Fla.)

2021 Volume 224, Page(s) 108676

Abstract: In this study, the potential of specific Circular RNAs (circRNAs) as novel peripheral blood biomarkers for myasthenia gravis (MG) was explored. We analyzed circRNAs in the peripheral blood of three normal controls and three MG patients using RNA ... ...

Abstract	In this study, the potential of specific Circular RNAs (circRNAs) as novel peripheral blood biomarkers for myasthenia gravis (MG) was explored. We analyzed circRNAs in the peripheral blood of three normal controls and three MG patients using RNA microarray. Candidate circRNAs were validated in three independent cohorts by Quantitative Real-time polymerase chain reaction (qPCR). Eleven differentially expressed circRNAs were initially identified and four were confirmed in the first independent cohort. Hsa_circ_0076490 and hsa-circ_5333-4 had the largest areas under the curve (AUCs) of the receiver operating characteristics (ROC) and were validated in the second cohort. In the third cohort, hsa-circRNA5333-4 had a larger AUC: 0.864 (95% confidence interval [CI] = 0.801-0.928, P < 0.001), a stronger correlation with the Quantitative Myasthenia Gravis Score (qMG): r = 0.505 (P < 0.001) and was correlated with gender and acetylcholine receptor antibody levels (P < 0.05). So hsa-circRNA5333-4 represents a novel biomarker for the diagnosis and monitoring of MG.
MeSH term(s)	Adolescent ; Adult ; Aged ; Aged, 80 and over ; Biomarkers/blood ; Child ; Child, Preschool ; Cohort Studies ; Female ; Gene Expression Regulation ; Humans ; Male ; Middle Aged ; Myasthenia Gravis/blood ; Myasthenia Gravis/genetics ; RNA, Circular/blood ; RNA, Circular/chemistry ; ROC Curve ; Reproducibility of Results ; Serum Albumin, Human/chemistry ; Young Adult
Chemical Substances	Biomarkers ; RNA, Circular ; Serum Albumin, Human (ZIF514RVZR)
Language	English
Publishing date	2021-01-17
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	1459903-x
ISSN	1521-7035 ; 1521-6616
ISSN (online)	1521-7035
ISSN	1521-6616
DOI	10.1016/j.clim.2021.108676
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Article: iTRAQ-Based Proteomics Analysis of Plasma of Myasthenia Gravis Patients Treated with Jia Wei Bu Zhong Yi Qi Decoction.

Zhang, Yunke / Yang, Junhong / Chen, Yingzhe / Lv, Jie / Zhang, Jing / Zhang, Yingna / Zhao, Xue / Fang, Hua / Liu, Chongchong / Zhang, Qingyong / Cui, Xinzheng / Wang, Xiaohan / Gao, Feng

Evidence-based complementary and alternative medicine : eCAM

2019 Volume 2019, Page(s) 9147072

Abstract: Myasthenia gravis (MG) is an autoimmune disease. A proportion of MG patients did not get satisfactory results after treatment with pyridostigmine and prednisone. Jia Wei Bu Zhong Yi Qi (Jia Wei BZYQ) decoction, a water extract from multiple herbs, has ... ...

Abstract	Myasthenia gravis (MG) is an autoimmune disease. A proportion of MG patients did not get satisfactory results after treatment with pyridostigmine and prednisone. Jia Wei Bu Zhong Yi Qi (Jia Wei BZYQ) decoction, a water extract from multiple herbs, has been demonstrated to be effective in the treatment of multiple "Qi deficiency type" diseases including MG in China. In this text, we investigated protein alterations in the plasma from healthy volunteers (C), MG patients without any treatment (T1), MG patients with routine western medical treatment (T2), and MG patients with combined treatments of Jia Wei BZYQ decoction and routine western medicines (T3) and identified some potential proteins involved in the pathogenesis and treatment of MG. iTRAQ (isobaric tags for relative and absolute quantitation) and 2D-LC-MS/MS (two-dimensional liquid chromatography-tandem mass spectrometry technologies) were employed to screen differentially expressed proteins. The identification, quantification, functional annotation, and interaction of proteins were analyzed by matching software and databases. In our project, 618 proteins were identified, among which 447 proteins had quantitative data. The number of differentially expressed proteins was 110, 117, 143, 115, 86, and 158 in T1 vs. C, T2 vs. C, T2 vs. T1, T3 vs. C, T3 vs. T1, and T3 vs. T2 groups, respectively. Functional annotation results showed that many differentially expressed proteins were closely associated with immune responses. For instance, some key proteins such as C-reactive protein, apolipoprotein C-III, apolipoprotein A-II, alpha-actinin-1, and thrombospondin-1 have been found to be abnormally expressed in T3 group compared to T1 group or T2 group. Interaction network analyses also provided some potential biomarkers or targets for MG management.
Language	English
Publishing date	2019-12-13
Publishing country	United States
Document type	Journal Article
ZDB-ID	2171158-6
ISSN	1741-4288 ; 1741-427X
ISSN (online)	1741-4288
ISSN	1741-427X
DOI	10.1155/2019/9147072
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Article ; Online: Clinical features of patients with Myasthenia gravis from the Henan province, China.

Gao, Feng / Zhao, Xue / Zhang, Jing / Cui, Xinzheng / Zhang, Yingna / Li, Qianru / Liu, Pingping / Fang, Hua / Du, Ying / Zhang, Qingyong / Yang, Junhong / Zhang, Yunke

Muscle & nerve

2016 Volume 53, Issue 5, Page(s) 711–716

Abstract: Introduction: Myasthenia gravis (MG) occurs globally, and many studies have indicated that there are regional differences in epidemiology, biomarkers, and prognosis of MG.: Methods: A total of 478 patients with MG who visited hospitals in the Henan ... ...

Abstract	Introduction: Myasthenia gravis (MG) occurs globally, and many studies have indicated that there are regional differences in epidemiology, biomarkers, and prognosis of MG. Methods: A total of 478 patients with MG who visited hospitals in the Henan Province between January 2010 and February 2014 were included. Age, gender, age at onset, serum antibody, thymus pathology, treatment information, and Myasthenia Gravis Foundation of America (MGFA) classification were assessed. Results: Compared with previous reports from other areas, we found some differences in MG patients from Henan. The proportion of childhood MG in our study was lower than other reports of Oriental patients with MG. There seems to be an association between onset age, muscle involvement, and acetylcholine receptor antibody levels. Thymectomy improved the symptoms in 78.5% of thymectomy-treated patients. Conclusions: These differences are likely related to geographical, environmental, and ethnic differences. Understanding these differences will help us to define more specific treatment.
MeSH term(s)	Adolescent ; Adrenal Cortex Hormones/therapeutic use ; Adult ; Age Distribution ; Age of Onset ; Aged ; Aged, 80 and over ; Autoantibodies/immunology ; Azathioprine/therapeutic use ; Child ; Child, Preschool ; China/epidemiology ; Cholinesterase Inhibitors/therapeutic use ; Cohort Studies ; Female ; Humans ; Immunoglobulins, Intravenous/therapeutic use ; Immunologic Factors/therapeutic use ; Immunosuppressive Agents/therapeutic use ; Infant ; Male ; Middle Aged ; Muscle Weakness ; Myasthenia Gravis/epidemiology ; Myasthenia Gravis/immunology ; Myasthenia Gravis/pathology ; Myasthenia Gravis/therapy ; Pyridostigmine Bromide/therapeutic use ; Receptors, Cholinergic/immunology ; Retrospective Studies ; Sex Distribution ; Thymectomy ; Thymus Gland/pathology ; Young Adult
Chemical Substances	Adrenal Cortex Hormones ; Autoantibodies ; Cholinesterase Inhibitors ; Immunoglobulins, Intravenous ; Immunologic Factors ; Immunosuppressive Agents ; Receptors, Cholinergic ; Pyridostigmine Bromide (KVI301NA53) ; Azathioprine (MRK240IY2L)
Language	English
Publishing date	2016-05
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	438353-9
ISSN	1097-4598 ; 0148-639X
ISSN (online)	1097-4598
ISSN	0148-639X
DOI	10.1002/mus.24920
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Article ; Online: Evaluation of the new classification and surgical strategy for myasthenia gravis.

Cui, Xin-Zheng / Ji, Xin-Ying / Gao, Feng / Yang, Kun-Peng / Bai, Hui-Ling / Ma, Hong-Bing / Li, Tao / Zhang, Qing-Yong

The American surgeon

2012 Volume 78, Issue 12, Page(s) 1329–1335

Abstract: The aim of this study was to discuss the new methods of clinical classification and staging of patients with myasthenia gravis (MG) proposed by our group and to summarize the experiences of surgical treatment of MG with a novel incision by cutting the ... ...

Abstract	The aim of this study was to discuss the new methods of clinical classification and staging of patients with myasthenia gravis (MG) proposed by our group and to summarize the experiences of surgical treatment of MG with a novel incision by cutting the sternum cross-sectionally at the second intercostal level. A retrospective analysis was made for the clinical data from the patients with MG who underwent thymectomy from July 1988 to May 2009. The surgical procedures were designed into three groups, a group with Osserman classification and median incision of the sternum (Group 1), a group with MGFA typing (Myasthenia Gravis Foundation of America) and a small transverse sternal incision at the second intercostal level (Group 2), and a group with new typing and a smaller transverse sternal incision at the second intercostal level (Group 3). Observation of the clinical typing and staging was made in the patients with myasthenia crisis. The parameters such as procedure duration in Group 2 and 3 was significantly lower than those in Group 1 (P < 0.05). The incidence of myasthenia crisis in Group 3 was significantly lower than that in Groups 2 and 3 (P < 0.05). The procedure with a smaller transverse sternal incision at the second intercostal level (Group 3) is a safer method for patients with MG. The combination of this procedure with the new typing and staging methods proposed by our group could facilitate the selection of operation indications and opportunity, resulting in the lower incidence of myasthenia crisis and mortality. Our new procedure is well deserved to be a preferential selection by other hospitals.
MeSH term(s)	Adolescent ; Adult ; Blood Loss, Surgical ; Cohort Studies ; Female ; Follow-Up Studies ; Humans ; Length of Stay ; Male ; Middle Aged ; Minimally Invasive Surgical Procedures/methods ; Myasthenia Gravis/classification ; Myasthenia Gravis/diagnosis ; Myasthenia Gravis/surgery ; Operative Time ; Postoperative Care/methods ; Preoperative Care/methods ; Retrospective Studies ; Risk Assessment ; Sternum/surgery ; Thymectomy/methods ; Thymoma/surgery ; Thymus Neoplasms/surgery ; Treatment Outcome ; Young Adult
Language	English
Publishing date	2012-12
Publishing country	United States
Document type	Comparative Study ; Evaluation Studies ; Journal Article
ZDB-ID	202465-2
ISSN	1555-9823 ; 0003-1348
ISSN (online)	1555-9823
ISSN	0003-1348
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