Pulmonary vascular lesions in end-stage idiopathic pulmonary fibrosis: Histopathologic study on lung explant specimens and correlations with pulmonary hemodynamics

Hum Pathol. 2007 Jan;38(1):60-5. doi: 10.1016/j.humpath.2006.06.007. Epub 2006 Aug 1.


In patients presenting with idiopathic pulmonary fibrosis (IPF), modifications of pulmonary vessels are well defined in fibrotic areas but have not been accurately assessed in the intervening patches of preserved lung. Moreover, the relation between pulmonary vessel lesions and pulmonary hemodynamics is not well known. We therefore designed a retrospective study on lung explant specimens from 26 patients with a firm diagnosis of IPF who had undergone lung transplantation. Our aim was to (1) describe the vascular lesions, especially in preserved lung areas, and (2) correlate them with pulmonary hemodynamics. In dense fibrotic zones, thickening of the arterial and venous wall with severe luminal narrowing was present in each patient. In architecturally preserved lung zones, occlusion of venules and small pulmonary veins was observed in 65% of the patients, although there were only mild changes of muscular pulmonary arteries. We found a significant positive correlation between the macroscopic extent of lung fibrosis and mean pulmonary artery pressure, but we failed to find a relation between mean pulmonary artery pressure and venous/venular lesions in nonfibrotic areas. Our study points out that in many patients with IPF, nonfibrotic lung areas demonstrate an occlusive venopathy, the signification of which remains undetermined.

MeSH terms

  • Adult
  • Aged
  • Female
  • Humans
  • Lung / blood supply
  • Lung / pathology*
  • Lung / physiopathology
  • Lung Transplantation
  • Male
  • Middle Aged
  • Pulmonary Artery / pathology
  • Pulmonary Artery / physiopathology
  • Pulmonary Circulation / physiology
  • Pulmonary Fibrosis / pathology*
  • Pulmonary Fibrosis / physiopathology
  • Pulmonary Fibrosis / surgery
  • Pulmonary Veins / pathology
  • Pulmonary Veins / physiopathology
  • Respiratory Function Tests
  • Retrospective Studies