TY - JOUR

T1 - The development of bronchiectasis on chest computed tomography in children with cystic fibrosis

T2 - can pre-stages be identified?

AU - Tepper, Leonie A.

AU - Caudri, Daan

AU - Rovira, Adria Perez

AU - Tiddens, Harm A. W. M.

AU - de Bruijne, Marleen

PY - 2016

Y1 - 2016

N2 - OBJECTIVE: Bronchiectasis is an important component of cystic fibrosis (CF) lung disease but little is known about its development. We aimed to study the development of bronchiectasis and identify determinants for rapid progression of bronchiectasis on chest CT.METHODS: Forty-three patients with CF with at least four consecutive biennial volumetric CTs were included. Areas with bronchiectasis on the most recent CT were marked as regions of interest (ROIs). These ROIs were generated on all preceding CTs using deformable image registration. Observers indicated whether: bronchiectasis, mucus plugging, airway wall thickening, atelectasis/consolidation or normal airways were present in the ROIs.RESULTS: We identified 362 ROIs on the most recent CT. In 187 (51.7 %) ROIs bronchiectasis was present on all preceding CTs, while 175 ROIs showed development of bronchiectasis. In 139/175 (79.4 %) no pre-stages of bronchiectasis were identified. In 36/175 (20.6 %) bronchiectatic airways the following pre-stages were identified: mucus plugging (17.7 %), airway wall thickening (1.7 %) or atelectasis/consolidation (1.1 %). Pancreatic insufficiency was more prevalent in the rapid progressors compared to the slow progressors (p = 0.05).CONCLUSION: Most bronchiectatic airways developed within 2 years without visible pre-stages, underlining the treacherous nature of CF lung disease. Mucus plugging was the most frequent pre-stage.KEY POINTS: • Development of bronchiectasis in cystic fibrosis lung disease on CT. • Most bronchiectatic airways developed within 2 years without pre-stages. • The most frequently identified pre-stage was mucus plugging. • This study underlines the treacherous nature of CF lung disease.

AB - OBJECTIVE: Bronchiectasis is an important component of cystic fibrosis (CF) lung disease but little is known about its development. We aimed to study the development of bronchiectasis and identify determinants for rapid progression of bronchiectasis on chest CT.METHODS: Forty-three patients with CF with at least four consecutive biennial volumetric CTs were included. Areas with bronchiectasis on the most recent CT were marked as regions of interest (ROIs). These ROIs were generated on all preceding CTs using deformable image registration. Observers indicated whether: bronchiectasis, mucus plugging, airway wall thickening, atelectasis/consolidation or normal airways were present in the ROIs.RESULTS: We identified 362 ROIs on the most recent CT. In 187 (51.7 %) ROIs bronchiectasis was present on all preceding CTs, while 175 ROIs showed development of bronchiectasis. In 139/175 (79.4 %) no pre-stages of bronchiectasis were identified. In 36/175 (20.6 %) bronchiectatic airways the following pre-stages were identified: mucus plugging (17.7 %), airway wall thickening (1.7 %) or atelectasis/consolidation (1.1 %). Pancreatic insufficiency was more prevalent in the rapid progressors compared to the slow progressors (p = 0.05).CONCLUSION: Most bronchiectatic airways developed within 2 years without visible pre-stages, underlining the treacherous nature of CF lung disease. Mucus plugging was the most frequent pre-stage.KEY POINTS: • Development of bronchiectasis in cystic fibrosis lung disease on CT. • Most bronchiectatic airways developed within 2 years without pre-stages. • The most frequently identified pre-stage was mucus plugging. • This study underlines the treacherous nature of CF lung disease.

U2 - 10.1007/s00330-016-4329-z

DO - 10.1007/s00330-016-4329-z

M3 - Journal article

C2 - 27108295

VL - 26

SP - 4563

EP - 4569

JO - European Radiology

JF - European Radiology

SN - 0938-7994

IS - 12

ER -