Pulmonary hypertension secondary to pulmonary fibrosis: clinical data, histopathology and molecular insights

Ruffenach, Grégoire; Hong, Jason; Vaillancourt, Mylène; Medzikovic, Lejla; Eghbali, Mansoureh

doi:10.1186/s12931-020-01570-2

Cited by 51 publications

(52 citation statements)

References 107 publications

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“…Several growth factors and signalling mechanisms, including transforming growth factor-β, hedgehog (SHH) and notch, are highly active in IPF tissues and are crucial to EndMT and proliferation [ 25 , 26 ]. Similar factors could be contributing to medial and adventitial thickening, for which smooth muscle hypertrophy and fibroblast hyperplasia were exhibited in our study of IPF arteries [ 23 , 27 ]. An interesting study by A rribas et al .…”

Section: Discussionsupporting

confidence: 71%

Vascular remodelling in IPF patients and its detrimental effect on lung physiology: potential role of endothelial to mesenchymal transition (EndMT)

Gaikwad

Dey

et al. 2022

ERJ Open Res

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BackgroundIPF is a progressive, irreversible fibrotic interstitial lung disease. We performed size-based quantitation of pulmonary arterial remodelling in IPF, examined the role of EndMT and effects on lung physiology.MethodsResected lung tissues from 11 normal controls (NC), and 13 IPF patients, were differentially stained using the Movat Pentachrome technique. Size-based classification for pulmonary arteries was conducted in NC and IPF tissues. For each pulmonary artery, arterial size, luminal diameter, thickness of the intima, media, adventitia, and elastin deposition was quantified using Image ProPlus7.0 software. In addition, immunohistochemical staining was performed for EndMT markers and collagen.ResultsLarge and medium size arterial numbers were significantly reduced in IPF compared to NC (p<0.0001). Intima thickness was highest in the arterial range of 200–399 μm and 600–1000 μm (p<0.0001), while medial and adventitial thickness was significant across 200–1000 μm (p<0.05) compared to NC. Medial thickness was found to significantly affect the diffusing capacity of the lungs for carbon monoxide (DLCO) (r’=−0.8, p=0.01). Total arterial elastin in IPF was higher across all arterial ranges except 100–199 μm in IPF than NC, with the greatest differences in 200–399 μm (p<0.001) and 600–1000 μm (p<0.001). Total elastin also negatively correlated with DLCO (r’=−0.63, p=0.04) in IPF. An increase in EndMT markers and collagen type I/ IV was observed.ConclusionsThis is the first study demonstrating size-based differences in pulmonary arteries in IPF and its detrimental effect on lung physiology. The process of EndMT might be central to these vascular remodelling changes and could be a potential novel therapeutic target.

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Section: Discussionsupporting

confidence: 71%

Vascular remodelling in IPF patients and its detrimental effect on lung physiology: potential role of endothelial to mesenchymal transition (EndMT)

Gaikwad

Dey

et al. 2022

ERJ Open Res

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“…In the current study, serum ANGP-2 concentration in patients with PH was significantly higher compared with that in patients with IPF without PH and that in age-matched HC. In the early stage of group 3 PH, hypoxic vasoconstriction and loss of vascular bed lead to the redistribution of pulmonary circulation 18 . In such a circumstance, the recruitment capacity and distensibility of pulmonary vessels play a role to keep a functional pulmonary circulation 19,20 .…”

Section: Discussionmentioning

confidence: 99%

Diagnostic and prognostic significance of serum angiopoietin-1 and -2 concentrations in patients with pulmonary hypertension

Enomoto

Suzuki

Hozumi

et al. 2021

Sci Rep

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Several biomarkers for detecting pulmonary hypertension (PH) have been reported. However, these biomarkers are deemed insufficient to detect PH in its early stages. We evaluated the utility of serum angiopoietin (ANGP), a glycoprotein related to angiogenesis, as a diagnostic and prognostic biomarker of PH. Patients with PH who underwent right-heart catheterization, were retrospectively studied. Serum concentrations of ANGP-1 and ANGP-2 were measured using an enzyme-linked immunosorbent assay in patients with PH (n = 32), those with idiopathic pulmonary fibrosis (IPF) without PH (as a disease control, n = 75), and age-matched healthy controls (HC, n = 60). Nineteen patients (59.4%) with PH had World Health Organization group 3 PH. Serum ANGP-2 concentration, but not ANGP-1, in patients with PH was significantly higher compared with that in HC (p = 0.025) and in patients with IPF without PH (p = 0.008). Serum ANGP-2 concentration in patients with PH positively and significantly correlated with N-terminal pro-B-type natriuretic peptide (r = 0.769, p < 0.001), right ventricular diameter on echocardiography (r = 0.565, p = 0.035), and mean pulmonary arterial pressure (r = 0.449, p = 0.032) and pulmonary vascular resistance (r = 0.451, p = 0.031) on right-heart catheterization. ANGP-1 and ANGP-2 were expressed on lung vascular endothelial cells, as shown by immunohistochemistry. Patients with PH with higher ANGP-2 concentration (≥ 2.48 ng/mL) had significantly worse survival (p = 0.022). Higher ANGP-2 concentration was a significant worse prognostic factor (hazard ratio = 6.063, p = 0.037), while serum ANGP-1 concentration was not. In conclusion, serum ANGP-2 may be a useful diagnostic and prognostic biomarker in patients with PH, especially in patients with group 3 PH.

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“…e587 CME: Respiratory medicine Pulmonary hypertension (PH) secondary to ILD is a frequent comorbidity and of concern because its presence is a strong predictor of mortality. 4 Patients can develop physiologically compromising right ventricular dysfunction and present with sudden breathlessness. Standard therapies for right heart failure apply, including optimising oxygen therapy to reduce pulmonary pressures and diuretics.…”

Section: Heart Failure Acute Pulmonary Embolism and Lung Cancer In The Patient With Ildmentioning

confidence: 99%

Interstitial lung disease on the acute take for the non-respiratory physician

2021

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Interstitial lung diseases (ILDs) are a heterogeneous group of diseases characterised by varying degrees of fibrotic and/ or inflammatory abnormalities of the lung parenchyma. Management of ILD is often challenging for non-respiratory physicians. We discuss the respiratory assessment and management of patients with ILD presenting with acute breathlessness on the acute take, including acute exacerbations of ILD.

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Pulmonary hypertension secondary to pulmonary fibrosis: clinical data, histopathology and molecular insights

Cited by 51 publications

References 107 publications

Vascular remodelling in IPF patients and its detrimental effect on lung physiology: potential role of endothelial to mesenchymal transition (EndMT)

Vascular remodelling in IPF patients and its detrimental effect on lung physiology: potential role of endothelial to mesenchymal transition (EndMT)

Diagnostic and prognostic significance of serum angiopoietin-1 and -2 concentrations in patients with pulmonary hypertension

Interstitial lung disease on the acute take for the non-respiratory physician

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