Síndrome de activación macrofágica como forma de presentación del lupus eritematoso sistémico en la infancia
Keywords:
síndrome de activación macrofágica, lupus eritematoso sistémico, relevancia clínica, reumatologíaAbstract
El síndrome de activación macrofágica se conoce como una forma de linfohistiocitosis hemofagocítica secundaria, relacionada con enfermedades autoinmunitarias. El objetivo de esta investigación fue presentar un caso de una paciente con síndrome de activación macrofágica, abordando el proceso diagnóstico, la estrecha relación con las enfermedades reumáticas, su seguimiento y tratamiento. Se presenta una paciente femenina de 11 años de edad con antecedentes de fiebre reumática, y el diagnóstico de SAM secundario a lupus eritematoso sistémico de debut. En el tratamiento de la paciente se emplearon esteroides a dosis elevadas e inmunosupresores, con lo cual se obtuvo una mejoría clínica y humoral, lográndose el egreso de la paciente. El síndrome de activación macrofágica, se mantiene como una entidad con elevada mortalidad, con potencialidad para provocar disfunción multiorgánica, que está relacionado fuertemente con diversas enfermedades reumatológicas, por lo que es imperativo que se piense y se diagnostique de forma temprana con el fin de disminuir la mortalidad y contribuir a engrandecer el conocimiento y su tratamiento.
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