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Case Report
Available online 24 January 2025
Autologous stem cell transplantation in systemic sclerosis: 4 cases
Trasplante autólogo de progenitores hematopoyéticos en esclerosis sistémica: 4 casos
Marta de la Rubia Navarro&#¿;
Corresponding author
mardena@mail.ucv.es

Corresponding author.
, Samuel Leal Rodríguez, José Andrés Román Ivorra
Servicio de Reumatología, Hospital Universitario y Politécnico La Fe, Valencia, Spain
Received 29 July 2024. Accepted 14 November 2024
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Table 1. Clinical-analytical patient data.
Abstract
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).

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.

Keywords:
Diffuse systemic sclerosis
Hematopoietic cell transplantation
Rodnan score
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.

Palabras clave:
Esclerosis sistémica difusa
Trasplante de médula ósea
Rodnan score

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