The different roles of the thymus in the pathogenesis of the various myasthenia gravis subtypes

Marx, Alexander; Pfister, Frederick; Schalke, Berthold; Saruhan‐Direskeneli, Güher; Melms, Arthur; Ströbel, Philipp

doi:10.1016/j.autrev.2013.03.007

Cited by 269 publications

(224 citation statements)

References 134 publications

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“…Approximately 70 % of patients with MG with anti-AChR antibodies have thymic follicular hyperplasia, approximately 10 % have thymomas, and the remainder have a histologically normal or atrophic thymus gland [4,13,14]. The alterations of the immune system that occur with thymic hyperplasia versus thymoma are quite distinct.…”

Section: Mg Subtypesmentioning

confidence: 97%

“…In the most common type of MG, autoantibodies are produced that target the skeletal muscle acetylcholine receptor (AChR), reducing the number of functional AChRs, and causing morphological damage to the endplate membrane, resulting in the clinical phenotype of fatigable muscle weakness [2]. The situation with AChR-associated MG becomes more complicated as there are clinical and immunological differences in patients with thymic abnormalities (thymic hyperplasia vs thymoma) versus no thymic pathology [3,4]. In the next most common form of MG, the immune target is the muscle-specific tyrosine kinase (MuSK), and the clinical disease, immunopathogenesis, and endplate pathology differ [5].…”

Section: Introductionmentioning

confidence: 99%

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Current Treatment, Emerging Translational Therapies, and New Therapeutic Targets for Autoimmune Myasthenia Gravis

2016

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Myasthenia gravis (MG) is an autoimmune disease associated with the production of autoantibodies against 1) the skeletal muscle acetylcholine receptor; 2) muscle-specific kinase, a receptor tyrosine kinase critical for the maintenance of neuromuscular synapses; 3) low-density lipoprotein receptor-related protein 4, an important molecular binding partner for muscle-specific kinase; and 4) other muscle endplate proteins. In addition to the profile of autoantibodies, MG may be classified according the location of the affected muscles (ocular vs generalized), the age of symptom onset, and the nature of thymic pathology. Immunopathologic events leading to the production of autoantibodies differ in the various disease subtypes. Advances in our knowledge of the immunopathogenesis of the subtypes of MG will allow for directed utilization of the ever-growing repertoire of therapeutic agents that target distinct nodes in the immune pathway relevant to the initiation and maintenance of autoimmune disease. In this review, we examine the pathogenesis of MG subtypes, current treatment options, and emerging new treatments and therapeutic targets.

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Section: Mg Subtypesmentioning

confidence: 97%

Section: Introductionmentioning

confidence: 99%

Current Treatment, Emerging Translational Therapies, and New Therapeutic Targets for Autoimmune Myasthenia Gravis

2016

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“…However, the accuracy of their information might be hampered by suboptimal antibody testing and evaluations of the thymus. MG with thymoma is considered as a paraneoplastic syndrome, which has different clinical and immunological features and different mechanisms compared with nonthymoma patients (5,11). Current guidelines of MG treatment do not recommend thymectomy in ocular MG and AChR antibody negative patients (12)(13)(14).…”

Section: Relative Precision In the Main Domains Of Myasthenia Gravis mentioning

confidence: 99%

“…Severity of MG was found to be correlated with antibody levels with conflicting evidence, partially due to the intrinsic mechanism of different antibodies, difference in methods of antibody testing, and the measurement of severity. Thymus abnormalities are often found in MG patients, with different presumed mechanism between hyperplasia and thymoma (5). Clinical and immunological heterogeneity exists in MG as a result of all these factors.…”

Section: Introductionmentioning

confidence: 99%

Precision medicine in myasthenia graves: begin from the data precision

Hong

Xie

et al. 2016

Ann. Transl. Med.

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Myasthenia gravis (MG) is a prototypic autoimmune disease with overt clinical and immunological heterogeneity. The data of MG is far from individually precise now, partially due to the rarity and heterogeneity of this disease. In this review, we provide the basic insights of MG data precision, including onset age, presenting symptoms, generalization, thymus status, pathogenic autoantibodies, muscle involvement, severity and response to treatment based on references and our previous studies. Subgroups and quantitative traits of MG are discussed in the sense of data precision. The role of disease registries and scientific bases of precise analysis are also discussed to ensure better collection and analysis of MG data.

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“…Aproximadamente 10% a 20% dos pacientes com MG apresentam timoma, e tipicamente esses pacientes apresentam fraqueza generalizada e ac-AChR positivo (Keesey, 2004;Marx et al, 2013).…”

Section: Introductionunclassified

Análise do jitter com agulha concêntrica em pacientes com miastenia gravis autoimune adquirida

Machado¹

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The different roles of the thymus in the pathogenesis of the various myasthenia gravis subtypes

Cited by 269 publications

References 134 publications

Current Treatment, Emerging Translational Therapies, and New Therapeutic Targets for Autoimmune Myasthenia Gravis

Current Treatment, Emerging Translational Therapies, and New Therapeutic Targets for Autoimmune Myasthenia Gravis

Precision medicine in myasthenia graves: begin from the data precision

Análise do jitter com agulha concêntrica em pacientes com miastenia gravis autoimune adquirida

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