La Evolución del Manejo de la Linfangioleiomiomatosis (LAM): De la Manipulación Hormonal a la Inhibición Dirigida de la Vía mammalian target of rapamycin (mTOR)
DOI:
https://doi.org/10.33448/rsd-v14i4.48656Palabras clave:
Linfangioleiomiomatosis, Inhibidores de mTOR, Enfermedades Raras, Sirolimus, Everolimus.Resumen
La Linfangioleiomiomatosis (LAM), una enfermedad pulmonar rara y progresiva que afecta predominantemente a mujeres, ha experimentado una significativa transformación en su manejo terapéutico en las últimas décadas. Esta revisión objetiva analizar críticamente dicha evolución, contrastando las estrategias históricas de manipulación hormonal con la terapia moderna dirigida a la inhibición de la vía mammalian target of rapamycin (mTOR), dilucidando la transición al estándar de tratamiento actual. Realizada como una revisión narrativa de la literatura científica, el análisis demuestra que los enfoques hormonales, a pesar de un racional biológico inicial que involucraba receptores e interacciones estrogénicas, no presentaron una eficacia robusta para modificar la progresión de la enfermedad pulmonar. El punto de inflexión ocurrió con la identificación de la vía mTOR como central en la patogénesis de la LAM, impulsada por mutaciones en los genes TSC. Este descubrimiento condujo al desarrollo y validación clínica de inhibidores de mTOR, como sirolimus y everolimus, que han demostrado estabilizar la función pulmonar y mejorar los resultados de las pacientes. Actualmente, los inhibidores de mTOR constituyen el estándar de oro terapéutico, representando un hito de la medicina de precisión en el tratamiento de la LAM. Al consolidar la evidencia que respalda este cambio de paradigma, esta revisión también destaca los desafíos persistentes y las direcciones para futuras investigaciones, incluyendo la optimización de la terapia, la identificación de nuevos biomarcadores y la búsqueda de tratamientos complementarios.
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