Fig. 2

Trajectory of pulmonary function before and after LEF treatment in entire cohort. a The FVC trajectory improved with treatment from a rate of 0.18 ± 1.90% (SEM) predicted/year to 4.62 ± 1.65%predicted/year. FVC% predicted increased significantly at 12 months (mean increase 4.4% predicted; 95% CI, 0.7 to 8.5%; p = 0.020). b DLCO was already improving at 0.87 ± 1.70% (SEM) predicted/year and continued to improve at 1.45 ± 1.44%predicted/year with LEF therapy. DLCO% predicted did not change significantly after 12 months of LEF treatment (mean increase 0.58% predicted; 95% CI, − 2.7 to 3.9%; p = 0.730)