Longitudinal Changes in Cardiac Function Following Autologous Stem Cell Transplantation in Diffuse Systemic Sclerosis.

Abstract

Cardiovascular involvement is frequent in patients with systemic sclerosis (SSc). Autologous stem-cell transplantation (ASCT) has emerged as a disease-modifying therapy for diffuse SSc, but its long-term impact on cardiac function remains unclear. We aim to examine the changes in left ventricular (LV) function after ASCT, and their potential prognostic significance. From the Leiden Systemic Sclerosis Cohort, 43 patients with diffuse SSc who underwent ASCT were matched 1:1 with SSc controls based on sex, age, and disease duration. Patients with overt cardiac or pulmonary involvement were excluded. Serial echocardiography was performed annually, including LV ejection fraction (LVEF), global longitudinal strain (GLS), and diastolic parameters. Among the included patients, mean age was 44.1±10.5 years; 52.3% were female. At baseline, ASCT patients showed mildly reduced LVEF (52.5±9.1% vs. 56.1±6.3%, P=0.044) and LV GLS (18.0±2.6% vs. 19.5±2.5%, P=0.014) compared to controls. During follow-up, ASCT was associated with significant improvement in LVEF (adjusted β 1.59%/year, P<0.001) and GLS (adjusted β 0.71%/year, P<0.001), whereas controls showed progressive decline. Diastolic parameters remained stable over time in both groups. Time-dependent changes in LV GLS, but not LVEF, were independently associated with all-cause mortality (adjusted HR 0.73 per %, 95% CI 0.61-0.86, P=0.0002). In patients with diffuse SSc, ASCT is associated with significant improvements in cardiac systolic function over time without adverse effects on diastolic function. These findings support the cardiac safety of ASCT and suggest potential cardiovascular benefit beyond systemic disease control, and highlight the importance of longitudinal monitoring with advanced echocardiography in SSc.