It is known that CFTR (cystic fibrosis transmembrane conductance regulator) modulators improve lung diseases associated with cystic fibrosis (CF). However, the effects of CFTR therapies on structural changes in the lungs of CF patients are unclear. US researchers therefore wanted to determine the effects on clinical parameters and structural lung disease, as measured by changes in chest CT scans, in people with CF.
Zu Beginn einer CFTR-Behandlung mit Elexacaftor/Tezacaftor/Ivacaftor (ETI) sowie über 1 Jahr lang in 3-monatigen Abständen wurden der prozentual vorhergesagte FEV1-Wert (ppFEV1), der BMI und mikrobiologische Daten erhoben, erläuterte Dr. Shahid I. Sheikh, pädiatrischer Pneumologe, Department of Pediatrics, Nationwide Children’s Hospital, Columbus, Ohio, in seiner Posterpräsentation [1]. CT-Aufnahmen des Brustkorbs wurden vor Beginn der ETI-Therapie (Baseline) und nach einem Jahr durchgeführt und von zwei Lungenfachärzten unabhängig voneinander analysiert.
The sample comprised 67 cystic fibrosis patients, 30 (44.8%) of whom were men, median age 25 years. After 3 months, a significant increase in ppFEV1 (65±21 to 77±21) and BMI (p<0.001 for both) and a significant decrease in MRSA and Pseudomonus positivity (-42% for both) were observed. This trend continued for over a year. None of the participants developed a deterioration in chest CT parameters during ETI therapy. At the start of treatment, the chest CT scans showed bronchiectasis in 65 patients (97%); at the follow-up examination after 1 year, it was no longer present or reduced in 17%. Bronchial wall thickening was detected at baseline in 64 (97%), after 12 months it was no longer present or had decreased in 58 (86%). Mucus congestion was found in 63 (95.5%), this was no longer present or had decreased in 61 (95%) after 1 year. At the beginning, 44 (67%) people had hyperinflation/air trapping; after 1 year, 38 (61%) had it less or not at all and 24 (39%) had it unchanged.
ETI therapy thus significantly improved clinical outcomes and lung disease as shown by chest CT scans, Dr. Sheikh and his colleagues concluded.
Source:
- Sheikh SI: Poster presentation 1541; ERS Congress 2023, Milan, 10.09.2023.
InFo PNEUMOLOGIE & ALLERGOLOGIE 2023; 5(4): 32
