Trasplante autólogo de progenitores hematopoyéticos en esclerosis sistémica: 4 casos

• de la Rubia Navarro, Marta ^[1] ; Leal Rodríguez, Samuel ^[1] ; Román Ivorra, José Andrés ^[1]
  1. [1] Servicio de Reumatología, Hospital Universitario y Politécnico La Fe, Valencia, Espa˜na
• Localización: Reumatología clínica, ISSN 1699-258X, Vol. 21, Nº. 1, 2025
• Idioma: español
• Títulos paralelos:
  • Autologous stem cell transplantation in systemic sclerosis: 4 cases
• Enlaces
  • Texto Completo Ejemplar
• Resumen
  • español

    Resumen Objetivo El objetivo es describir las características clínicas, serológicas, los tratamientos previos/actuales y los eventos adversos de 4 pacientes con esclerosis sistémica (ES) que se han sometido a un auto-TPH. Material y métodos Estudio descriptivo, transversal de los pacientes con ES (criterios ACR/EULAR 2013). Se recogieron los tratamientos previos y actuales, eventos adversos y datos clínicos/serológicos (2014-2023). Resultados Se incluyeron 4 mujeres (edad media de 49 [6,78 años) y mediana de 32,5 [7 meses] desde el diagnóstico hasta el auto-TPH. El m-Rodnan score (mRSS) medio fue de 33,75. En todos los casos se produjo una mejoría (diferencia media de 14,75) y la función pulmonar (DLCO) se mantuvo estable. Solo una paciente presentó una neumonía tras el trasplante, requiriendo ingreso en la unidad de cuidados intensivos, con mejoría y resolución completa del cuadro infeccioso. Conclusiones El auto-TPH es una estrategia terapéutica adecuada en los pacientes con diagnóstico de ES difusa rápidamente progresiva, con un buen perfil de seguridad en nuestra cohorte. Objective The aim is to describe clinical and serological features, previous and current therapies, and adverse events in four systemic sclerosis patients (SSc) who undergo autologous hematopoietic stem cells transplantation (AHSCT). Material and methods Descriptive, cross-sectional study including SSc patients according ACR/EULAR 2013 criteria. Clinical and serological data, data related to current and previous therapy and adverse events were collected from 2014 to 2023. Results Four female patients were included, with a mean age of 49 (6.78) years old and a mean of 32.5 (7) months since diagnosis to AHSCT. Mean mRodnan score (mRSS) was 33.75. All cases underwent a skin improvement measured by mRSS (mean difference of 14.75) and lung function (DLCO) remained stable. Only one patient presented a pneumonia post-AHSCT, that required admission to intensive care unit, with improvement and complete resolution of infection a few days after admission. Conclusions AHSCT is an appropriate therapeutic option in rapidly progressive diffuse SSc patients, with a good safety profile in our cohort. .

  • English

    The aim is to describe clinical and serological features, previous and current therapies, and adverse events in four systemic sclerosis patients (SSc) who undergo autologous hematopoietic stem cells transplantation (AHSCT).

    Material and methods: Descriptive, cross-sectional study including SSc patients according ACR/EULAR 2013 criteria. Clinical and serological data, data related to current and previous therapy and adverse events were collected from 2014 to 2023.

    Results: Four female patients were included, with a mean age of 49 (6.78) years old and a mean of 32.5 (7) months since diagnosis to AHSCT. Mean mRodnan score (mRSS) was 33.75. All cases underwent a skin improvement measured by mRSS (mean difference of 14.75) and lung function (DLCO) remained stable. Only one patient presented a pneumonia post-AHSCT, that required admission to intensive care unit, with improvement and complete resolution of infection a few days after admission.

    Conclusions: AHSCT is an appropriate therapeutic option in rapidly progressive diffuse SSc patients, with a good safety profile in our cohort