Myasthenia gravis (MG) is a rare autoimmune disease, hallmarked by pathogenic autoantibodies targeting the neuromuscular junction (NMJ). The antibodies affect AChR densities and neuromuscular architecture, which impairs neuromuscular transmission and causes the cardinal symptom of fatigable muscle weakness that can be life-threatening if respiratory muscles are affected. MG is a heterogeneous disease with diverging age of onset, disease severity, treatment responses, thymic pathologies, and autoantibody constellations.
Our understanding of antibody-mediated autoimmunity has been significantly shaped by studying the immunopathology of MG. It has become a textbook case for B-cell mediated and T-cell dependent autoimmunity. Despite being the prototype of an antibody-mediated neurological disease, treatment options in MG were, for many years, limited to symptomatic treatment, corticosteroids, and long term-immunosuppressants over decades. In recent years, the therapeutic landscape of MG has changed profoundly including novel immunomodulatory therapies like C5-Inihibitors, FcRn-inhibitors, and most recently CAAR T-cells which stimulates further research in this area. However, novel therapies with monoclonal antibodies are mainly limited to the treatment of “classical” AChR-ab-positive MG, and specific subgroups (e.g. ocular MG, seronegative MG) are largely underrepresented in medical research.
Understanding the underlying pathogenetic mechanisms of the clinical heterogeneity of MG including the combination of genetic susceptibilities, environmental factors and defects in the immune system might pave the way toward patient-oriented, individualized treatment strategies in MG.
In this current Research Topic of Frontiers in Immunology, our goal is to bridge clinical and basic research. We welcome the submission of articles that cover, but not limited to, the following sub-topics:
• The Immunobiology of MG
• Characteristics and diagnostic challenges of autoantibodies
• Clinical management
• Novel treatment options in MG
Myasthenia gravis (MG) is a rare autoimmune disease, hallmarked by pathogenic autoantibodies targeting the neuromuscular junction (NMJ). The antibodies affect AChR densities and neuromuscular architecture, which impairs neuromuscular transmission and causes the cardinal symptom of fatigable muscle weakness that can be life-threatening if respiratory muscles are affected. MG is a heterogeneous disease with diverging age of onset, disease severity, treatment responses, thymic pathologies, and autoantibody constellations.
Our understanding of antibody-mediated autoimmunity has been significantly shaped by studying the immunopathology of MG. It has become a textbook case for B-cell mediated and T-cell dependent autoimmunity. Despite being the prototype of an antibody-mediated neurological disease, treatment options in MG were, for many years, limited to symptomatic treatment, corticosteroids, and long term-immunosuppressants over decades. In recent years, the therapeutic landscape of MG has changed profoundly including novel immunomodulatory therapies like C5-Inihibitors, FcRn-inhibitors, and most recently CAAR T-cells which stimulates further research in this area. However, novel therapies with monoclonal antibodies are mainly limited to the treatment of “classical” AChR-ab-positive MG, and specific subgroups (e.g. ocular MG, seronegative MG) are largely underrepresented in medical research.
Understanding the underlying pathogenetic mechanisms of the clinical heterogeneity of MG including the combination of genetic susceptibilities, environmental factors and defects in the immune system might pave the way toward patient-oriented, individualized treatment strategies in MG.
In this current Research Topic of Frontiers in Immunology, our goal is to bridge clinical and basic research. We welcome the submission of articles that cover, but not limited to, the following sub-topics:
• The Immunobiology of MG
• Characteristics and diagnostic challenges of autoantibodies
• Clinical management
• Novel treatment options in MG