Novel therapeutic monoclonal against B cells at different stages of their maturation (like plasmablasts), or against molecules involved in B cell activation, represent promising therapeutic targets. Reduction of antibody titers follows the clinical efficacy of anti-CD20 therapies, a feature implying the role of short-lived plasma cells (SLPB) in autoantibody production. Exceptional clinical response to plasmapheresis and rituximab has been particularly observed in these patients. IVIG) with the need for prolonged and high doses of steroids difficult to be tapered to control symptoms. In MuSK-MG, classical treatment options are usually less effective (e.g. Cardinal differences between AChR and MuSK-MG are the thymus involvement (not prominent in MuSK-MG), the distinct HLA alleles, and core immunopathological patterns of pathology in neuromuscular junction, structure, and function. IgG4 class autoantibodies are impotent to fix complement and only weakly bind Fc-receptors expressed on immune cells and exert their pathogenicity via interfering with the interaction between their targets and binding partners (e.g. On the other hand, acetylcholine receptor (AChR) MG, the most prevalent form of MG, is characterized by immunoglobulin (Ig)G1 and IgG3 antibodies to the AChR. The pathogenic antibodies mostly belong to the immunoglobulin subclass (Ig)G4, a feature which attributes them their specific properties and pathogenic profile. Muscle-specific kinase (MuSK) Myasthenia Gravis (MG) represents a prototypical antibody-mediated disease characterized by predominantly focal muscle weakness (neck, facial, and bulbar muscles) and fatigability. 9Department of Neurology, The University of Tennessee Health Science Center, Memphis, TN, United States.8Department of Neurology, Henry Dunant Hospital Center, Athens, Greece. 7Department of Pharmacy, University of Patras, Patras, Greece.6Department of Neurobiology, Hellenic Pasteur Institute, Athens, Greece.5Tzartos NeuroDiagnostics, Athens, Greece.4Department of Physiotherapy, University of West Attica, Athens, Greece.3Department of Neurology, School of Medicine, University of Patras, Patras, Greece.2Second Department of Neurology, Attikon University Hospital, National and Kapodistrian University of Athens, Athens, Greece.1First Department of Neurology, Medical School, National and Kapodistrian University of Athens, Athens, Greece.Vakrakou 1† Eleni Karachaliou 2† Elisabeth Chroni 3 Vasiliki Zouvelou 1 Dimitrios Tzanetakos 2 Stavroula Salakou 2 Marianna Papadopoulou 2,4 Socrates Tzartos 5,6,7 Konstantinos Voumvourakis 2 Constantinos Kilidireas 1,8 Sotirios Giannopoulos 2 Georgios Tsivgoulis 2,9 John Tzartos 2*
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