PT - JOURNAL ARTICLE AU - Stijn E. Verleden AU - Arno Vanstapel AU - Laurens De Sadeleer AU - Birgit Weynand AU - Matthieu Boone AU - Erik Verbeken AU - Davide Piloni AU - Dirk Van Raemdonck AU - Maximilian Ackermann AU - Danny D. Jonigk AU - Johny Verschakelen AU - Wim A. Wuyts TI - Quantitative analysis of airway obstruction in lymphangioleiomyomatosis AID - 10.1183/13993003.01965-2019 DP - 2020 Jul 01 TA - European Respiratory Journal PG - 1901965 VI - 56 IP - 1 4099 - http://erj.ersjournals.com/content/56/1/1901965.short 4100 - http://erj.ersjournals.com/content/56/1/1901965.full SO - Eur Respir J2020 Jul 01; 56 AB - Lymphangioleiomyomatosis (LAM) is a rare, cystic lung disease with progressive pulmonary function loss caused by progressively proliferating LAM cells. The degree of airway obstruction has not been well investigated within the pathogenesis of LAM.Using a combination of ex vivo computed tomography (CT), microCT and histology, the site and nature of airway obstruction in LAM explant lungs was compared with matched control lungs (n=5 each). The total number of airways per generation, total airway counts, terminal bronchioles number and surface density were compared in LAM versus control.Ex vivo CT analysis demonstrated a reduced number of airways from generation 7 on (p<0.0001) in LAM compared with control, whereas whole-lung microCT analysis confirmed the three- to four-fold reduction in the number of airways. Specimen microCT analysis further demonstrated a four-fold decrease in the number of terminal bronchioles (p=0.0079) and a decreased surface density (p=0.0079). Serial microCT and histology images directly showed the loss of functional airways by collapse of airways on the cysts and filling of the airway by exudate.LAM lungs show a three- to four-fold decrease in the number of (small) airways, caused by cystic destruction which is the likely culprit for the progressive loss of pulmonary function.This study demonstrates a 4-fold reduction in the number of airways and terminal bronchioles in end-stage LAM lungs using a combination of CT, microCT and histopathology, compared to a matched control group http://bit.ly/2tBTiJy