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Inicio Medicina Clínica (English Edition) Myasthenia gravis. Update on diagnosis and therapy
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Vol. 161. Issue 3.
Pages 119-127 (August 2023)
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Vol. 161. Issue 3.
Pages 119-127 (August 2023)
Review
Myasthenia gravis. Update on diagnosis and therapy
Miastenia gravis. Actualización diagnóstica y terapéutica
Daniel Apolinar García Estéveza,b,
,1
, Julio Pardo Fernándezc,1
a Servicio de Neurología, Complexo Hospitalario Universitario de Ourense, Ourense, Spain
b Grupo de investigación Neurociencias Clínicas, Instituto de Investigaciones Sanitarias Galicia-Sur, SERGAS-UVIGO, Vigo, Pontevedra, Spain
c Unidad de Enfermedades Neuromusculares, Servicio de Neurología, Complexo Hospitalario Universitario de Santiago de Compostela, Facultad de Medicina, Universidad de Santiago de Compostela, Santiago de Compostela, A Coruña, Spain
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Tables (4)
Table 1. Clinical classification according to the Myasthenia Gravis Foundation of America.26
Table 2. Classification of myasthenia gravis by autoantibody subgroups.
Table 3. Differential diagnosis of myasthenia gravis.
Table 4. Chronic medical treatments in myasthenia gravis.
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Abstract

Myasthenia gravis is an autoimmune disease caused by the presence of specific antibodies targeting different postsynaptic components of the neuromuscular junction, and is clinically characterized by the presence of fatigueable muscle weakness. In the etiopathogenesis plays a central role the thymus and the most frequently detected pathogenic autoantibodies are targeted to the acetylcholine receptor. The increase in the knowledge of the immunological components of the neuromuscular junction in the last two decades has been fundamental to identify new pathogenic antibodies, reduce the percentage of patients with seronegative myasthenia, and propose a classification of patients into subgroups with clinical-therapeutic interest. In addition, in recent years, new drugs have been developed for the treatment of patients with myasthenia gravis that are refractory to conventional immunosuppressive treatment.

Keywords:
Myasthenia gravis
Autoantibodies
Thymus
Acetylcholine receptor
Neuromuscular junction
Immunotherapy
Resumen

La miastenia gravis es una enfermedad autoinmune causada por la presencia de anticuerpos específicos dirigidos a diferentes componentes postsinápticos de la unión neuromuscular, y clínicamente se caracteriza por la presencia de debilidad muscular fatigable. En la etiopatogenia juega un papel central el timo, y los autoanticuerpos patogénicos más frecuentemente detectados están dirigidos al receptor de la acetilcolina. El incremento en el conocimiento de los componentes inmunológicos de la unión neuromuscular en las últimas dos décadas ha sido fundamental para identificar nuevos anticuerpos patogénicos, reducir el porcentaje de pacientes con miastenia seronegativa y proponer una clasificación de los pacientes en subgrupos con interés clínico-terapéutico. Además, en los últimos años hemos asistido al desarrollo de nuevos fármacos destinados al tratamiento de los pacientes con miastenia que muestran refractariedad al tratamiento inmunosupresor convencional.

Palabras clave:
Miastenia gravis
Autoanticuerpos
Timo
Receptor de acetilcolina
Unión neuromuscular
Inmunoterapia

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