Skip to main content
Log in

Heterogeneous Remodeling of Lung Vessels in Idiopathic Pulmonary Fibrosis

  • Published:
Lung Aims and scope Submit manuscript

Abstract

Recently, several reports suggest differences in the vascularization of the various histopathologic patterns of parenchymal remodeling seen in usual interstitial pneumonia (UIP). In this study, we sought to validate the importance of vascular remodeling in patients with idiopathic pulmonary fibrosis (IPF) and to examine the relationship between vascular remodeling and parenchymal remodeling or pulmonary function. Open lung biopsies were performed in 57 patients with IPF, and vascular changes in alternating areas of parenchymal remodeling (UIP histologic patterns) were studied. Quantitative analysis of the internal area, internal perimeter, wall thickness, and surrounding cellularity of medium or large pulmonary arteries, as well as their distribution according to air/parenchymal ratios, was performed. Semiquantitative analysis also was used to determine the grade of vascular occlusion. An inverse association was found between vascularization and UIP parenchymal remodeling (p < 0.05); that is, the decreased internal luminal area and perimeter as well as the increased wall thickness run in parallel with progression from alveolar collapse toward severe mural-organizing fibrosis with honeycombing. Vascular regression (diminished internal area and perimeter of vessels) was also associated with higher FEV1, FVC, and RV values (r = 0.48, p< 0.05), reflecting a tight relationship between vascular remodeling and pulmonary function. A progressive regression of vascularization, reflected by different degrees of luminal occlusion after vascular remodeling, coincided with parenchymal remodeling (alveolar collapse, mural-organizing fibrosis, and honeycombing). This vascular regression may be responsible for the impaired wound healing and progressive fibroproliferation found in patients with IPF. Further studies are needed to determine whether this relationship is causal or consequential.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2.

Similar content being viewed by others

References

  1. American Thoracic Society (2000) Idiopathic Pulmonary Fibrosis: diagnosis and treatment. International Consensus Statement. Am J Respir Crit Care Med 161:646–664

    Google Scholar 

  2. American Thoracic Society/European Respiratory Society (2002) International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonia. Am J Respir Grit Care Med 165:277–304

    Google Scholar 

  3. Basset F, Ferrans VJ, Soler P, Takemura T, Fukuda Y, Crystal RG (1986) Intraluminal fibrosis in interstitial lung disorders. Am J Pathol 122:443–461

    PubMed  Google Scholar 

  4. Coalson JJ (1982) The ultrastructure of human fibrosing alveolitis. Virchows Arch A Pathol Anat Hist 395:181–199

    Article  Google Scholar 

  5. Cutaia M, Rounds S (1990) Hypoxic pulmonary vasoconstriction: physiologic significance, mechanism, and clinical relevance. Chest 97:706–718

    PubMed  Google Scholar 

  6. Demedts M, Costabel U (2002) ATS/ERS international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Eur Respir J 19:794–796

    Article  PubMed  Google Scholar 

  7. Fukuda Y, Ferrans VJ, Schoenberger CI, Rennard SI, Crystal RG (1985) Patterns of pulmonary structural remodeling after experimental paraquat toxicity. The morphogenesis of intraalveolar fibrosis. Am J Pathol 118:452–475

    PubMed  Google Scholar 

  8. Fukuda Y, Ishizaki M, Masuda Y, Kimura G, Kawanami O, Masugi Y (1987) The role of intraalveolar fibrosis in the process of pulmonary structural remodeling in patients with diffuse alveolar damage. Am J Pathol 126:171–182

    PubMed  Google Scholar 

  9. Heath D, Edwards JE (1958) The pathology of hypertension pulmonary vascular disease. A description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac defects. Circulation 18:533–547

    PubMed  Google Scholar 

  10. Katzenstein AL (1985) Pathogenesis of “fibrosis” in interstitial pneumonia. An electron microscopic study. Hum Pathol 16:1015–1024

    PubMed  Google Scholar 

  11. Katzenstein AL, Myers JL, Mazur MT (1986) Acute interstitial pneumonia A clinicopathologic, ultrastructural, and cell kinetic study. Am J Respir Grit Care Med 10:256–267

    Google Scholar 

  12. Katzenstein AL, Myers JL (1998) Idiopathic pulmonary fibrosis. Clinical relevance of pathologic classification. Am J Respir Crit Care Med 157:1301–1315

    PubMed  Google Scholar 

  13. Norusis MJ (2002) SPSS for Windows [10.0]. SPSS Inc., Chicago, IL

    Google Scholar 

  14. Peao MND, Aguas AP, DeSa CM, Grande NR (1994) Neoformation of blood vessels in association with rat lung fibrosis induced by bleomycin. Anat Rec 238:57–67

    Article  PubMed  Google Scholar 

  15. Renzoni EA, Walsh DA, Salmon M, Wells AU, Sestini P, Nicholson AG, et al (2003) Interstitial vascularity in fibrosing alveolitis. Am J Respir Crit Care Med 167:438–443

    Article  PubMed  Google Scholar 

  16. Salmon M, Lui YC, Mark JC, Rousell J, Huang TJ, Hisada T, et al (2000) Contribution of upregulation airway endothelin–1 expression to airway smooth muscle and epithelial cell DNA synthesis after repeated allergen exposure of sensitized Brown–Norway rats. Am J Respir Cell Mol Biol 23:618–625

    PubMed  Google Scholar 

  17. Turner–Warwick M (1963) Precapillary systemic-pulmonary anastomoses. Thorax 18:225–237

    PubMed  Google Scholar 

  18. Wiebe BM, Laursen H (1998) Lung morphometry by unbiased methods in emphysema: bronchial and blood vessel volume, alveolar surface area and capillary length. APMIS 106(6):651–656

    PubMed  Google Scholar 

Download references

Acknowledgments

This study was supported by the following Brazilian agencies: the National Council for Scientific and Technological Development [CNPq 300430/95-7]; the Foundation for the Support of Research of the State of São Paulo [FAPESP 2000/14336-0, 2001/14566-9 and 2003/00162-9]; and the Laboratories for Medical Research [LIM 05], Clinicas Hospital, School of Medicine, University of São Paulo.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Vera Luiza Capelozzi.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Parra, E.R., David, Y.R., Costa, L.R.S.d. et al. Heterogeneous Remodeling of Lung Vessels in Idiopathic Pulmonary Fibrosis. Lung 183, 291–300 (2005). https://doi.org/10.1007/s00408-004-2542-z

Download citation

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00408-004-2542-z

Keywords

Navigation