Figure 4.

Ivacaftor reduces inflammatory cytokines but does not prevent the response to LPS. hG551D rats were treated with methylcellulose vehicle or ivacaftor for 7 days, and BALF collected. Ivacaftor reduced (A) total protein concentration in the BALF, as well as concentrations of (B) IFN-γ, (C) IL-6, (D) TNF-α, (E) IL-1α, and (F) IL-1β. Rats pretreated with methylcellulose or ivacaftor and stimulated with LPS had (G) reduced protein concentration in the BALF, as well as concentrations of (H) IFN-γ and (L) IL-1β that were reduced, whereas concentrations of (I) IL-6, (J) TNF-α, and (K) IL-1α were unchanged. Data presented as mean ± SEM. *P < 0.05, **P < 0.01, ***P < 0.001, ****P < 0.0001. BALF, bronchoalveolar lavage fluid; hG551D, homozygous G551D; LPS, lipopolysaccharide; WT, wild type.