Background/Purpose: Myocardial involvement in systemic sclerosis (SSc) represents a severe complication with a poor prognosis; however, compromised cardiac function also heightens the risk of life-threatening complications associated with the most effective treatment for SSc, autologous stem cell transplantation (ASCT). In the current study we evaluated the evolution of cardiac function after ASCT in SSc, with the goal of improving patient selection for ASCT. The central dilemma is whether patients with myocardial involvement should or should not be treated with ASCT, given the potential risks.

Methods: Consecutive SSc patients from the Leiden Combined care in Systemic Sclerosis prospective cohort study undergoing ASCT were included and matched 1:1 with controls using propensity scores based on sex, age and disease duration. Echocardiography was performed before ASCT and then annually: left ventricular ejection fraction (LVEF), left ventricular global longitudinal strain (LV-GLS), and diastolic function parameters were measured per guidelines. Linear mixed-effect models were used to analyze their changes over time.

Results: A total of 86 patients (n=43 per each group) were included. Clinical characteristics at baseline were comparable between the groups and during follow-up n=15 patients died (n=8 controls) (Table 1). LVEF and LV-GLS were comparable between ASCT patients and controls at baseline, but significantly improved (LVEF: β 1.59%, 95% confidence interval CI [0.84, 2.34], interaction p< 0.001; LV-GLS: β -0.71%, 95% CI [-0.43, -0.99], interaction p< 0.001) in the ASCT group at thei third year and at last follow-up, while worsened over time in the controls (Figure). In turn, LV diastolic function parameters, including EA ratio, tricuspid regurgitation velocity, E/e’, and left atrial volume index showed no significant changes over time in both groups.

Conclusion: The study shows that ASCT, despite the chemotherapy involved, significantly benefits SSc patients in terms of LV systolic function, evidenced by improvements in LVEF and LV-GLS over time. This observation indicates that SSc patients with cardiac involvement should not be excluded from ASCT. Additional safety monitoring by using echocardiographic measures such as LV-GLS, or offering ASCT as an early treatment option might be effective strategies in this patient group.This study also highlights the role of LV-GLS as a potential marker for monitoring myocardial response to this treatment, making it a valuable tool in clinical practice for assessing cardiac damage and treatment efficacy in SSc patients undergoing ASCT.

Baseline characteristics of SSc patients included in the study

Figure A: Course of LVEF (Left Ventricular Ejection fraction) over time in SSc-controls (Non-ASCT) and SSc patients treated with ASCT (ASCT)

Figure B: Course of LV-GLS (Left Ventricular Global Longitidinal Strain) over time in SSc-controls (Non-ASCT) and SSc patients treated with ASCT (ASCT)

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/impact-of-autologous-stem-cell-transplantation-on-cardiac-performance-in-systemic-sclerosis/