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

Colombat, Magali; Mal, Hervé; Groussard, O; Capron, Frédérique; Thabut, Gabriel; Jébrak, G.; Brugière, Olivier; Dauriat, Gaëlle; Castier, Yves; Lesèche, Guy; Fournier, Michel

doi:10.1016/j.humpath.2006.06.007

Cited by 125 publications

(91 citation statements)

References 14 publications

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“…[2][3][4] They suggest that when considered in the light of these previous reports, our findings add support to the idea that vascular alterations might be the first pathological lesion in IPF preceding, and leading to, fibrogenesis.…”

supporting

confidence: 87%

Impact of pulmonary vascular volume on mortality in IPF: is it time to reconsider the role of vasculature in disease pathogenesis and progression?

et al. 2017

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supporting

confidence: 87%

Impact of pulmonary vascular volume on mortality in IPF: is it time to reconsider the role of vasculature in disease pathogenesis and progression?

et al. 2017

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“…22,23,26 Furthermore, vasoactive mediators such as endothelin-1, platelet-derived growth factor, transforming growth factor-b , and fi broblast growth factor have all been implicated in the pathogenesis of IPF, and these mediators can also contribute to the development of lung injury. [27][28][29][30] In addition to raising questions about the shared pathophys iologic characteristics of secondary PAH and PGD, our study may have direct clinical implications. Knowledge of elevated mPAP as an independent risk factor for PGD in patients with IPF may help in counseling patients and families on the risks of transplantation.…”

Section: Discussionmentioning

confidence: 99%

Elevated Pulmonary Artery Pressure Is a Risk Factor for Primary Graft Dysfunction Following Lung Transplantation for Idiopathic Pulmonary Fibrosis

Fang

Studer

Kawut

et al. 2011

Chest

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Idiopathic pulmonary fibrosis (IPF) is a fatal and chronically progressive diffuse parenchymal lung disease that is characterized by varying degrees of fi brosis of the lung parenchyma. 1 Currently, there are few proven, standardized therapies for IPF 2 ; the only viable treatment that has shown survival benefi t for patients with advanced stages of IPF is lung transplantation. [3][4][5][6][7] Nonetheless, the survival benefi t for lung transplantation in patients with IPF is still complicated by signifi cant early mortality rates, especially within the fi rst year (approximately 28%). The 10-year survival rate after lung transplantation is worse for patients with IPF when compared with other diseases, such as idiopathic pulmonary arterial hypertension (PAH), cystic fi brosis, and a 1 -antitrypsin-defi ciency emphysema. 8 Elevated mean pulmonary arterial pressure (mPAP) in patients with IPF has been associated with decreased exercise capacity and worse survival. 9 Higher mPAP was also associated with increased mortality following lung transplantation in a large registry study. 10 One possible explanation for the link between PAH and early mortality after lung transplantation

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“…In areas of dense fibrosis, sharp decreases in vessel density are observed [25,26]. The walls of pulmonary arteries show intimal fibrosis and medial hypertrophy, and are thickened [27,28]. Pulmonary veins and venules present loose and intimal fibrosis with reduced calibre [28].…”

Section: Pathobiology and Pathology Of Ipfmentioning

confidence: 99%

Physiology of the lung in idiopathic pulmonary fibrosis

et al. 2018

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The clinical expression of idiopathic pulmonary fibrosis (IPF) is directly related to multiple alterations in lung function. These alterations derive from a complex disease process affecting all compartments of the lower respiratory system, from the conducting airways to the lung vasculature. In this article we review the profound alterations in lung mechanics (reduced lung compliance and lung volumes), pulmonary gas exchange (reduced diffusing capacity, increased dead space ventilation, chronic arterial hypoxaemia) and airway physiology (increased cough reflex and increased airway volume), as well as pulmonary haemodynamics related to IPF. The relative contribution of these alterations to exertional limitation and dyspnoea in IPF is discussed.

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

Cited by 125 publications

References 14 publications

Impact of pulmonary vascular volume on mortality in IPF: is it time to reconsider the role of vasculature in disease pathogenesis and progression?

Impact of pulmonary vascular volume on mortality in IPF: is it time to reconsider the role of vasculature in disease pathogenesis and progression?

Elevated Pulmonary Artery Pressure Is a Risk Factor for Primary Graft Dysfunction Following Lung Transplantation for Idiopathic Pulmonary Fibrosis

Physiology of the lung in idiopathic pulmonary fibrosis

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