Distinct Airway Involvement in Subtypes of End-Stage Fibrotic Pulmonary Sarcoidosis.
Aged
Belgium
Bronchiectasis
/ diagnostic imaging
Disease Progression
Female
Humans
Lung Diseases, Interstitial
/ diagnostic imaging
Lung Transplantation
Male
Middle Aged
Pulmonary Fibrosis
/ diagnostic imaging
Sarcoidosis, Pulmonary
/ diagnostic imaging
Tomography, X-Ray Computed
X-Ray Microtomography
airway
imaging
interstitial lung disease
lung fibrosis
pathology
sarcoidosis
Journal
Chest
ISSN: 1931-3543
Titre abrégé: Chest
Pays: United States
ID NLM: 0231335
Informations de publication
Date de publication:
08 2021
08 2021
Historique:
received:
18
08
2020
revised:
17
11
2020
accepted:
04
01
2021
pubmed:
14
1
2021
medline:
4
1
2022
entrez:
13
1
2021
Statut:
ppublish
Résumé
Sarcoidosis is a systemic granulomatous disease that in most patients affects the lung. Pulmonary fibrotic sarcoidosis is clinically, radiologically, and pathologically a heterogeneous condition. Although substantial indirect evidence suggests small airways involvement, direct evidence currently is lacking. What is the role of the (small) airways in fibrotic sarcoidosis? Airway morphologic features were investigated in seven explant lungs with end-stage fibrotic sarcoidosis using a combination of CT scanning (large airways), micro-CT scanning (small airways), and histologic examination and compared with seven unused donor lungs as controls with specific attention focused on different radiologically defined sarcoidosis subtypes. Patients with central bronchial distortion (n = 3), diffuse bronchiectasis (n = 3), and usual interstitial pneumonia pattern (n = 1) were identified based on CT scan, showing a decrease and narrowing of large airways, a similar airway number and increased airway diameter of more distal airways, or an increase in airway number and airway diameter, respectively, compared with control participants. The number of terminal bronchioles per milliliter and the total number of terminal bronchioles were decreased in all forms of fibrotic sarcoidosis. Interestingly, the number of terminal bronchioles was inversely correlated with the degree of fibrosis. Furthermore, we identified granulomatous remodeling as a cause of small airways loss using serial micro-CT scanning and histologic examination. The large airways are involved differentially in subtypes of sarcoidosis, but the terminal bronchioles universally are lost. This suggests that small airways loss forms an important aspect in the pathophysiologic features of fibrotic pulmonary sarcoidosis.
Sections du résumé
BACKGROUND
Sarcoidosis is a systemic granulomatous disease that in most patients affects the lung. Pulmonary fibrotic sarcoidosis is clinically, radiologically, and pathologically a heterogeneous condition. Although substantial indirect evidence suggests small airways involvement, direct evidence currently is lacking.
RESEARCH QUESTION
What is the role of the (small) airways in fibrotic sarcoidosis?
STUDY DESIGN AND METHODS
Airway morphologic features were investigated in seven explant lungs with end-stage fibrotic sarcoidosis using a combination of CT scanning (large airways), micro-CT scanning (small airways), and histologic examination and compared with seven unused donor lungs as controls with specific attention focused on different radiologically defined sarcoidosis subtypes.
RESULTS
Patients with central bronchial distortion (n = 3), diffuse bronchiectasis (n = 3), and usual interstitial pneumonia pattern (n = 1) were identified based on CT scan, showing a decrease and narrowing of large airways, a similar airway number and increased airway diameter of more distal airways, or an increase in airway number and airway diameter, respectively, compared with control participants. The number of terminal bronchioles per milliliter and the total number of terminal bronchioles were decreased in all forms of fibrotic sarcoidosis. Interestingly, the number of terminal bronchioles was inversely correlated with the degree of fibrosis. Furthermore, we identified granulomatous remodeling as a cause of small airways loss using serial micro-CT scanning and histologic examination.
INTERPRETATION
The large airways are involved differentially in subtypes of sarcoidosis, but the terminal bronchioles universally are lost. This suggests that small airways loss forms an important aspect in the pathophysiologic features of fibrotic pulmonary sarcoidosis.
Identifiants
pubmed: 33440183
pii: S0012-3692(21)00025-8
doi: 10.1016/j.chest.2021.01.003
pii:
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
562-571Informations de copyright
Copyright © 2021 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.