help button home button
AJRCCM
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by DURIEU, I.
Right arrow Articles by PACHECO, Y.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by DURIEU, I.
Right arrow Articles by PACHECO, Y.

Am. J. Respir. Crit. Care Med., Volume 158, Number 2, August 1998, 580-588

Subepithelial Fibrosis and Degradation of the Bronchial Extracellular Matrix in Cystic Fibrosis

ISABELLE DURIEU, SIMONE PEYROL, DOMINIQUE GINDRE, GABRIEL BELLON, DENIS VITAL DURAND, and YVES PACHECO

Departments of Internal Medicine, Pneumology, and Pediatrics, Centre Hospitalier Lyon-Sud; and Department of Cellular Pathology, Institut Pasteur, Lyon, France

Cystic fibrosis is a genetic disease caused by mutations of the cystic fibrosis transmembrane conductance regulator gene. Chronic inflammation and proteolysis lead to progressive damage of the bronchial wall. Extracellular matrix determines the structural organization and the mechanical properties of lung airways. It was thus examined in nine patients with cystic fibrosis (six bronchial biopsies and three lobectomies) in order to assess its level of alteration. The submucosal changes in matrix protein distribution were analyzed by immunochemistry and electron microscopy: the subepithelial basal lamina was thinned; an acellular collagen fiber layer composed of interstitial collagens (types I and III) subtended by tenascin and devoid of elastin-associated microfibrils was deposited beneath the basal lamina; this dense fibrous deposit generally formed a thick layer and could extend into the bronchial wall; the bronchial elastic framework lost arborescent distribution and appeared slender, packed, or lacunar; ultrastructural observation gave evidence for elastic and collagenic fiber lysis. Proteolytic activity is probably the major cause of matrix degradation. Fibrosis appears as a repair process rather than as an active fibrogenesis. The reversibility of extracellular matrix alterations is an important challenge and various interventions such as anti-inflammatory treatments can be targeted to halt or reverse this degradation process.




This article has been cited by other articles:


Home page
 J Intensive Care MedHome page
T. M. Kremer, R. G. Zwerdling, P. H. Michelson, and B. P. O'Sullivan
Intensive Care Management of the Patient With Cystic Fibrosis
J Intensive Care Med, May 1, 2008; 23(3): 159 - 177.
[Abstract] [PDF]

Home page
 ThoraxHome page
T. N Hilliard, N. Regamey, J. K Shute, A. G Nicholson, E. W F W Alton, A. Bush, and J. C Davies
Airway remodelling in children with cystic fibrosis
Thorax, December 1, 2007; 62(12): 1074 - 1080.
[Abstract] [Full Text] [PDF]

Home page
 Eur Respir JHome page
A. L. James and S. Wenzel
Clinical relevance of airway remodelling in airway diseases
Eur. Respir. J., July 1, 2007; 30(1): 134 - 155.
[Abstract] [Full Text] [PDF]

Home page
 Eur Respir JHome page
C. Bergeron, M. K. Tulic, and Q. Hamid
Tools used to measure airway remodelling in research
Eur. Respir. J., March 1, 2007; 29(3): 596 - 604.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Respir. Cell Mol. Bio.Home page
Y. Tesfaigzi
Roles of Apoptosis in Airway Epithelia
Am. J. Respir. Cell Mol. Biol., May 1, 2006; 34(5): 537 - 547.
[Abstract] [Full Text] [PDF]

Home page
 ChestHome page
C. Bergeron and L.-P. Boulet
Structural changes in airway diseases: characteristics, mechanisms, consequences, and pharmacologic modulation.
Chest, April 1, 2006; 129(4): 1068 - 1087.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Respir. Crit. Care Med.Home page
R. L. Gibson, J. L. Burns, and B. W. Ramsey
Pathophysiology and Management of Pulmonary Infections in Cystic Fibrosis
Am. J. Respir. Crit. Care Med., October 15, 2003; 168(8): 918 - 951.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Respir. Crit. Care Med.Home page
P. Jeffery, S. Holgate, and S. Wenzel
Methods for the Assessment of Endobronchial Biopsies in Clinical Research: Application to Studies of Pathogenesis and the Effects of Treatment
Am. J. Respir. Crit. Care Med., September 15, 2003; 168(6): S1 - 17.
[Full Text] [PDF]

Home page
 Am. J. Respir. Crit. Care Med.Home page
H. TIDDENS, M. SILVERMAN, and A. BUSH
The Role of Inflammation in Airway Disease . Remodeling
Am. J. Respir. Crit. Care Med., August 1, 2000; 162(2): S7 - 10.
[Full Text] [PDF]

Home page
 Am. J. Respir. Crit. Care Med.Home page
A. BUSH and P. POHUNEK
Brush Biopsy and Mucosal Biopsy
Am. J. Respir. Crit. Care Med., August 1, 2000; 162(2): S18 - 22.
[Full Text] [PDF]

Home page
 ThoraxHome page
I. Durieu, C. Amsellem, C. Paulin, M.-T. Chambe, J. Bienvenu, G. Bellon, and Y Pacheco
Fas and Fas ligand expression in cystic fibrosis airway epithelium
Thorax, December 1, 1999; 54(12): 1093 - 1098.
[Abstract] [Full Text]


HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
Proc. Am. Thorac. Soc. Am. J. Respir. Cell Mol. Biol.
Copyright © 1998 American Thoracic Society