Quantitative analysis of airway obstruction in lymphangioleiomyomatosis.
Eur Respir J
; 56(1)2020 07.
Article
en En
| MEDLINE
| ID: mdl-32108050
ABSTRACT
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.
Texto completo:
1
Bases de datos:
MEDLINE
Asunto principal:
Linfangioleiomiomatosis
/
Obstrucción de las Vías Aéreas
/
Neoplasias Pulmonares
Tipo de estudio:
Etiology_studies
Límite:
Humans
Idioma:
En
Revista:
Eur Respir J
Año:
2020
Tipo del documento:
Article
País de afiliación:
Bélgica