Current and emerging therapies for the treatment of myasthenia gravis

Abstract

Myasthenia gravis (MG) is an autoimmmune disease in which autoantibodies to different antigens of the neuromuscular junction cause the typical weakness and fatigability. Treatment includes anticholinesterase drugs, immunosuppression, immunomodulation, and thymectomy. The autoimmune response is maintained under control by corticosteroids frequently associated with immunosuppressive drugs, with improvement in the majority of patients. In case of acute exacerbations with bulbar symptoms or repeated relapses, modulation of autoantibody activity by plasmapheresis or intravenous immunoglobulins provides rapid improvement. Recently, techniques removing only circulating immunoglobulins have been developed for the chronic management of treatment-resistant patients. The rationale for thymectomy relies on the central role of the thymus. Despite the lack of controlled studies, thymectomy is recommended as an option to improve the clinical outcome or promote complete remission. New videothoracoscopic techniques have been developed to offer the maximal surgical approach with the minimal invasiveness and hence patient tolerability. The use of biological drugs such as anti-CD20 antibodies is still limited but promising. Studies performed in the animal model of MG demonstrated that several more selective or antigen-specific approaches, ranging from mucosal tolerization to inhibition of complement activity or cellular therapy, might be feasible. Investigation of the transfer of these therapeutic approaches to the human disease will be the challenge for the future.

Miastenia gravis de tipo bulbar en niños: un caso de difícil diagnóstico.

“…La prednisona y la azatioprina, como fármacos inmunosupresores, que interfieren con la actividad de auto anticuerpos en la unión neuromuscular, y la inmunoglobulina que disminuye los autoanticuerpos circulantes. La timectomía modifica la historia natural de la enfermedad (12,29,30) por ello es obligatoria en pacientes con timoma y es recomendado como opción para los pacientes con MG generalizada sin timoma, en particular aquellos con anticuerpos de acetilcolina y menores de 60 años, porque aumenta la probabilidad de mejoría o remisión (31).…”

The Study of Dynamics of Clinical and Molecular Phenotypes in the Generalized Form of Myasthenia With Optimized and Combined Treatment

“…Главными направлениями терапии миастении в насто-ящее время является применение антихолинэстеразных средств (АХЭП), неспецифических иммуносупрессантов, тимэктомия, плазмаферез [11][12][13]. Однако лечение со-провождается большим числом нежелательных побочных реакций, таких как аритмии, остеопороз и гипотензия, в результате чего не может быть достигнута полная ре-миссия заболевания [14,15].…”

Acetylcholine Receptor-Induced Experimental Myasthenia Gravis: What Have We Learned from Animal Models After Three Decades?

“…Current pharmacological therapies for the treatment of MG include steroids and immunosuppressants, as long-term drugs, and immunomodulating agents, as short-term therapy (Mantegazza et al 2011). Azathioprine and cyclophosphamide provided successful results in the treatment of experimental MG in rabbits (Abramsky et al 1976) and rats (Pestronk et al 1983), respectively.…”