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Title
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Quantitative analysis of airway obstruction in lymphangioleiomyomatosis
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Author
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Abstract
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| 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. |
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Language
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English
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Source (journal)
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The European respiratory journal / European Respiratory Society [Lausanne] - Copenhagen, 1988, currens
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Publication
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Copenhagen
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2020
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ISSN
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0903-1936
[print]
1399-3003
[online]
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DOI
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10.1183/13993003.01965-2019
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Volume/pages
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56
:1
(2020)
, 11 p.
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Article Reference
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1901965
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ISI
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000558984300013
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Pubmed ID
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32108050
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Medium
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E-only publicatie
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Full text (Publisher's DOI)
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