Reduction of systemic inflammation by elexacaftor/tezacaftor/ivacaftor correlates with lung function improvement in cystic fibrosis

BACKGROUND: Triple CFTR modulator therapy elexacaftor/tezacaftor/ivacaftor (ETI) rapidly improves airway and systemic inflammation in people with cystic fibrosis (CF). However, longitudinal effects on systemic inflammation and their relationship to lung function remain unknown. METHODS: In this prospective, observational, multicenter study, we analyzed peripheral blood neutrophil counts, C-reactive protein (CRP) and six pro-inflammatory serum cytokines in a cohort of 198 people with CF aged 6 years and older at baseline (BL) and follow-up visits 3, 12, and 24 months after initiation of ETI, compared to 74 age-matched healthy control participants (HCs). RESULTS: Neutrophil counts and CRP, G-CSF, IL-1

• Halle, O.
• Graeber, S. Y.
• Kontsendorn, J.
• Kessemeier, C.
• Falke, J. N.
• Schwabe, J.
• Schütz, K.
• Pallenberg, S. T.
• Dalferth, R.
• Grychtol, R.
• Ringshausen, F. C.
• Stahl, M.
• Thee, S.
• Roehmel, J. F.
• Syunyaeva, Z.
• Duerr, J.
• Chung, J.
• Hirtz, S.
• Uselmann, T.
• Kühbandner, I.
• Rückes-Nilges, C.
• Bagheri-Pothoff, A.
• Barth, S.
• Schaub, B.
• Brinkmann, F.
• Weber, S.
• Koningsbruggen-Rietschel, S. V.
• Abdo, M.
• Weckmann, M.
• Widder, S.
• Hansen, G.
• Tümmler, B.
• Sommerburg, O.
• Naehrlich, L.
• Mall, M. A.
• Dittrich, A. M.