Conclusion
The extent of structural damage to the lungs of CF patients is
correlated with the response in ppFEV1 on ivacaftor,
however this correlation was not demonstrable in patients starting on
lumacaftor/ivacaftor. It would take a larger drug effect size and/or
larger sample size to see if this correlation also stands after
correction for baseline ppFEV1. Brody score derived from
chest CT in CF patients correlates with ppFEV1 before
the start of CFTR modulating treatment.