Vol. 18 Núm. 3 (2024): Revista Reumatología al Día
Artículos Originales

Síndrome del pulmón encogido en el lupus eritematoso sistémico: Una entidad poco frecuente. Reporte de 6 casos.

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  • J. J. Astudillo Andrade,
  • M. Micelli,
  • V. Cosentino,
  • E. Kerzberg
J. J. Astudillo Andrade
Servicio de Reumatología y Colagenopatías. Hospital J. M. Ramos Mejía; CABA, Argentina.
DOI: https://doi.org/10.62484/rad.v18i3.9257

Publicado 24-12-2024

Palabras clave

  • síndrome de pulmón encogido,
  • lupus eritematoso sistémico,
  • disnea inexplicable,
  • patrón ventilatorio restrictivo

Cómo citar

Astudillo Andrade, J. J., Micelli, M., Cosentino, V., & Kerzberg, E. (2024). Síndrome del pulmón encogido en el lupus eritematoso sistémico: Una entidad poco frecuente. Reporte de 6 casos. Reumatología Al Día, 18(3), 9. https://doi.org/10.62484/rad.v18i3.9257

Derechos de autor 2024 Reumatología al Día

Creative Commons License

Esta obra está bajo una licencia internacional Creative Commons Atribución-NoComercial-SinDerivadas 4.0.

Resumen

Una de las manifestaciones respiratorias poco frecuente en el Lupus Eritematoso sistémico (LES) es el síndrome del pulmón encogido (SPE), clínicamente caracterizado por disnea inexplicable, dolor pleurítico y una característica triada: reducción del volumen pulmonar, elevación diafragmática y defecto ventilatorio restrictivo.

Objetivo: describir las características clínicas de una serie de 6 pacientes con diagnóstico de SPE asociado a LES y su relación con la actividad y daño.

Método: e studio r etrospectivo, c on d atos o btenidos d e h istorias clínicas. Se recolectaron datos demográficos, clínicos, serológicos y radiológicos al diagnóstico y a los 6 meses.

Resultados: De 220 pacientes con diagnóstico de LES, 6 cumplían con todos los criterios de SPE, calculando una prevalencia de 2.7%; 4 fueron mujeres, con una media de edad de 31.3 (+-9.5) años; el tiempo entre el debut de LES y SPE fue de 72 (+-67.7) meses. Al momento del diagnóstico, el 100% tuvieron disnea y ortopnea; las manifestaciones no respiratorias concomitantes más frecuentes fueron: rash malar (83.3%), artritis, artralgias y linfopenia (66.6%). Todos los pacientes (100%) se interpretaron como activos por presentar un SLEDAI 2K >4. A los 6 meses del diagnóstico, el 66.7% estaban inactivos, pero el daño por la enfermedad según el SLICC/ACR SDI aumento en el 100% de los pacientes.

Conclusiones: Ante la presencia de disnea inexplicable, debemos pensar en patologías poco frecuentes como el SPE. Relacionar su presencia con la actividad de la enfermedad es importante y es necesario tener siempre la sospecha de esta patología.

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