Changes on chest HRCT in systemic sclerosis-related interstitial lung disease after autologous haematopoietic stem cell transplantation

Abstract

Objective To evaluate extent of interstitial lung disease (ILD) and esophageal involvement using high-resolution computed tomography (HRCT) in early diffuse systemic sclerosis (dSSc)-patients after autologous hematopoietic stem cell transplantation (aHSCT). Methods Overall chest HRCT, lung function and skin score changes were evaluated in 33 consecutive dSSc-patients before and after aHSCT during yearly routine follow-up visits between January 2000 and September 2016. Two independent radiologists blindly assessed the ILD extent using semi-quantitative Goh and Wells method, the widest esophageal diameter (WED) and the esophageal volume (EV) on HRCT. Patients were retrospectively classified as radiological responders or non-responders based on achieved stability or a decrease of 5% or more of HRCT-ILD at 24 months post-aHSCT. Results Using a linear mixed model, the regression of the extent of ILD and of ground glass opacities were significant at 12 months (ILD p= 0.001; ground glass opacities p= 0.0001) and at 24 months (ILD p= 0.007; ground glass opacities p= 0.0008) after aHSCT, with 18 patients classified as radiological responders (probability of response 0.78, 95% CI (0.58; 0.90), meanwhile the WED and the EV increased significantly at 12 months (WED p= 0.03; EV p= 0.34) and at 24 months (WED p= 0.002; EV p= 0.007). Kaplan-Meier analyses showed a trend towards better 5-year survival rates (100% vs 60%; HR 0.23 95%CI 0.03–1.62, p= 0.11) among radiological responders vs non-responders at 24 months follow-up. Conclusion Real-world data analysis confirmed significant improvement in extent of HRCT SSc-ILD 24 months after aHSCT, although esophageal dilatation worsened requiring specific attention.