TRAPS, síndrome periódico asociado al receptor de necrosis tumoral: inmunopatogénesis y enfoque clínico

  • Catalina Moreno-Zuluaga Universidad de Antioquia.
  • Margarita María Velásquez-Lopera Universidad de Antioquia
Palabras clave: Fiebre, Inflamación, Factor de Necrosis Tumoral Alfa, Receptores del Factor de Necrosis Tumoral

Resumen

El síndrome periódico asociado al receptor del factor de necrosis tumoral (TRAPS), se caracteriza por episodios de fiebre de más de 10 días de duración, mialgias migratorias, pseudocelulitis, dolor abdominal y edema bipalpebral. Su principal complicación es la amiloidosis y la falla renal producida por el estado inflamatorio crónico. Es una enfermedad autosómica dominante por mutación en el gen TNFRSF1A que codifica el receptor 1 del factor de necrosis tumoral (TNF). En las hipótesis elaboradas para explicar la enfermedad se describen la alteración en la liberación del receptor de TNF mutado, la activación del factor nuclear potenciador de las cadenas ligeras kappa de las células B activadas (NFkB) de forma independiente, las proteínas mal plegadas y alteraciones en el tráfico del receptor mutado, llevando a la acumulación de especies reactivas del oxígeno y defectos en la muerte celular por autofagia y apoptosis. Se han dirigido muchos esfuerzos en descubrir las bases inmunopatogénicas del TRAPS, dificultado por el elevado número de mutaciones encontradas, que se traducen en diferentes mecanismos y formas de presentación de la enfermedad. El tratamiento se basa en el bloqueo del TNF y de la interleuquina-1 (IL-1), la mejor comprensión de la inmunopatogénesis podría permitir un mejor seguimiento de los pacientes y el empleo de otras terapias.

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Biografía del autor/a

Catalina Moreno-Zuluaga, Universidad de Antioquia.

Médica, Residente de Dermatología. Universidad de Antioquia

Margarita María Velásquez-Lopera, Universidad de Antioquia

Dermatóloga, Doctora en ciencias básicas biomédicas, Inmunología, Profesora, Sección de Dermatología. Centro de Investigaciones Dermatológicas, CIDERM Universidad de Antioquia.

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Publicado
2018-10-01
Cómo citar
1.
Moreno-Zuluaga C, Velásquez-Lopera MM. TRAPS, síndrome periódico asociado al receptor de necrosis tumoral: inmunopatogénesis y enfoque clínico. Iatreia [Internet]. 1 de octubre de 2018 [citado 27 de septiembre de 2021];31(4):380-92. Disponible en: https://revistas.udea.edu.co/index.php/iatreia/article/view/329192
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