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Desarrollo de autoinmunidad en pacientes con inmunodeficiencias primarias / Development of autoimmunity in patients with primary immunodeficiencies
Resumen
Resumen
Las enfermedades autoinmunes constituyen un grupo de trastornos del sistema inmunológico en dónde este ataca a las células propias del organismo. Las causas pueden ser multifactoriales y no hay tratamientos específicos contra estas enfermedades. Por su parte, las inmunodeficiencias primarias (IDP) son un grupo de alteraciones originadas por defectos genéticos que tienen como consecuencia la deficiencia en la función del sistema inmunológico. Actualmente, se han descrito algunos mecanismos celulares y moleculares por los cuales se desarrollan trastornos autoinmunes en pacientes con inmunodeficiencias, sin embargo, dichos mecanismos no se han descrito con exactitud. Lo anterior, representa uno de los principales retos de las personas que lo padecen. De manera interesante, diversos reportes indican que la autoinmunidad secundaria a la inmunodeficiencia sigue algunos mecanismos celulares y moleculares como: una deficiente maduración de células inmunológicas; deficiencia de proteínas importantes para la función de los linfocitos T y B y; fallas en la función de moléculas de señalización intracelular importantes para la regulación inmunológica. En conjunto, estos mecanismos se relacionan con el desarrollo de autoinmunidad en pacientes con IDP. El objetivo del presente trabajo fue realizar una revisión de la evidencia disponible hasta la fecha respecto a los mecanismos celulares y moleculares implicados en el desarrollo de autoinmunidad en pacientes con IDP.
Abstract
The autoimmune diseases include many in which the immune system is directed against the host, leading to life-threatening destruction of organs. The origin of autoimmune disorders can be multifactorial and, there are no specific therapy for these diseases. Primary immunodeficiencies are a group of immune disorders that affect different components of the innate and adaptive responses. Interestingly, patients with primary immunodeficiencies have an increased susceptibility to infectious diseases and non-infectious complications including allergies, malignancies, and autoimmune diseases. The molecular mechanism for development of autoimmunity in immunodeficiencies is unclear. The study of the complex immune regulatory and signaling mechanisms is revealing the relationships between primary immunodeficiency syndromes and autoimmune diseases. Newly, it has been demonstrated that a deficient maturation of immune cells; the deficiency of proteins important for T and B lymphocyte function and impaired signally pathways that include key molecules in regulation and activation of immune cells are associated with the development of autoimmunity in patients with primary immunodeficiencies. The aim of the present work is to review the evidence available to date regarding the cellular and molecular mechanisms involved in the development of autoimmunity in patients with primary immunodeficiencies.
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