Table 1.
Demographic data and ILD characteristics at baseline.
Fig 1.
Evolution of FVC during the follow up.
Panel A represents the individual data of the 75 patients and the red line is the trajectory of FVC using the linear mixed model with random effect. There was no significant change of FVC over time. Panel B is the progression-free survival of FVC. Progression was defined as a decline of ≥10% of baseline FVC.
Fig 2.
Evolution of DLCO during the follow up.
Panel A represents the individual data of the 75 patients and the red line is the trajectory of DLCO using the linear mixed model with random effect. DLCO significantly decreased over time (-1.5±0.3%/year (p<0.0001). Panel B is the progression-free survival of DLCO. Progression was defined as a decline of ≥15% of baseline DLCO.
Table 2.
Bivariate analysis of parameters associated with baseline value and slope of DLCO.
Fig 3.
Trajectories of DLCO of patients with various clinical characteristics, using the linear mixed model with random effect.
Panel A. Patients with presence or history of digital ulcers (DU) had a significantly higher baseline DLCO% but a faster decline of DLCO than patients without presence or history of digital ulcers (no DU) in multivariate analysis: -2.45±0.45 vs -1.01±0.33%/year (p = 0.01). Panel B. Patients with a right heart catheterization-proven precapillary pulmonary hypertension at baseline or during follow-up (PH) had a similar baseline DLCO% but a faster decline of DLCO than patients without a right heart catheterization-proven precapillary pulmonary hypertension at baseline or during follow-up (no PH) in multivariate analysis: -2.37±0.55 vs -1.19±0.31%/year (p = 0.049). Panel C. Patients with a limited ILD according to Goh et al. had a significantly higher baseline DLCO than patients with an extensive ILD but the slope of decrease in DLCO was similar in both groups (-1.69±0.33 vs -0.90±0.53%/year (p = 0.21). Panel D. Patients with a limited cutaneous SSc (lcSSc) had similar baseline DLCO and slope of decrease in DLCO than patients with diffuse SSc (dSSc) (p = 0.40 and p = 0.93, respectively).