Effects of ivacaftor on systemic inflammation and the plasma proteome in people with CF and G551D

•Ivacaftor led to sustained reductions in serum HMGB-1 and calprotectin.•SAA and G-CSF decreased and IGF-1 increased 1 month after treatment initiation.•Proteomic analyses identified 9 proteins that changed significantly with ivacaftor.•Ivacaftor altered lipid digestion/transport and extracellular matrix organization proteins.•These changes lend insight into the extrapulmonary effects of CFTR modulation.