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

Jacob, Joseph; Nicholson, Andrew G.; Wells, Athol U.; Hansell, David M.

doi:10.1183/13993003.02524-2016

Cited by 8 publications

(5 citation statements)

References 10 publications

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“… 7 , 8 There is a growing appreciation for the importance of the vasculature in the disease process, including possibly being important in the perpetuation of the fibrosis. 9 In support of this, two studies of PH medications have suggested amelioration of the fibrotic process as evidenced by placebo‐corrected improvements in the forced vital capacity. 10 , 11 …”

Section: Introductionmentioning

confidence: 92%

“…Pulmonary hypertension (PH) frequently complicates the course of patients with IPF and is associated with a myriad of adverse consequences including higher oxygen needs, reduced functional ability, increased healthcare resource utilization, and increased mortality 7,8 . There is a growing appreciation for the importance of the vasculature in the disease process, including possibly being important in the perpetuation of the fibrosis 9 . In support of this, two studies of PH medications have suggested amelioration of the fibrotic process as evidenced by placebo‐corrected improvements in the forced vital capacity 10,11 …”

Section: Introductionmentioning

confidence: 99%

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Pulmonary vascular dysfunction without pulmonary hypertension: A distinct phenotype in idiopathic pulmonary fibrosis

Nathan,

Tehrani,

Zhao

et al. 2024

Pulm. circ.

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Pulmonary vascular dysfunction in the absence of pulmonary hypertension (PH) has been observed in patients with idiopathic pulmonary fibrosis (IPF). We describe the prevalence and etiology of elevated pulmonary vascular resistance (PVR) without PH among patients with IPF. Hemodynamic, echocardiographic, and functional respiratory imaging (FRI) data was compared between patients with IPF without PH with normal (<3 wood units) and elevated PVR (≥3 wood units). Mortality between these two groups were compared to patients with IPF and PH. Of 205 patients with IPF, there were 146 patients without PH, of whom 114 (78.1%) had a normal PVR and 32 (21.9%) who had a high PVR. Functional testing and hemodynamics were similar in the two groups, except for the cardiac index which was significantly lower in patients with a high PVR (2.3 vs. 2.6 L/min/m2; p = 0.004). Echocardiographic comparison demonstrated a higher tricuspid regurgitant velocity in those with a high PVR (3.4 vs 3.0 m/s; p = 0.046). FRI revealed proportionately fewer large vessels as a proportion of the vasculature in the patients without PH and elevated PVRs. Among patients without PH, PVR was associated with increased mortality. In conclusion, patients with IPF without PH but a high PVR appear to be a distinct phenotype with a prognosis between those with and without PH, likely reflecting the continuum of vascular dysfunction. The basis for this unique hemodynamic profile could not be definitively discerned although FRI suggested an aberrant anatomical vascular response.

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Section: Introductionmentioning

confidence: 92%

Section: Introductionmentioning

confidence: 99%

Pulmonary vascular dysfunction without pulmonary hypertension: A distinct phenotype in idiopathic pulmonary fibrosis

Nathan,

Tehrani,

Zhao

et al. 2024

Pulm. circ.

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“…Cross-sectional area of subsegmental small pulmonary vessels has been shown to correlate with the extent of CT density measures of emphysema and reflects difference between COPD phenotypes [37,38]. The volume of segmented pulmonary vessels, including arteries and veins but excluding vessels at the hilum, expressed as a percentage of lung volume has been proposed as an independent measure of IPF severity [39][40][41]. Beyond structure perfusion can be assessed using dual-energy CT.…”

Section: Blood Vesselsmentioning

confidence: 99%

“…The assessment of the completeness or integrity of fissures, as a biomarker for collateral flow, was first applied in a subgroup analysis of subjects undergoing endobronchial valve (EBV) placement for treatment of emphysema [45]. In this prespecified analysis, patients with a fissure integrity score >90% of the fissure abutting the treatment lobe were shown to have significant improvement in their forced expiratory volume in 1 s (FEV1) compared to those whose FIS was <90% [41]. The majority of automated fissure detection methods use feature descriptors targeted toward normal fissure anatomy [46][47][48][49][50][51][52][53][54][55][56].…”

Section: Fissuresmentioning

confidence: 99%

The Emerging Role of Quantification of Imaging for Assessing the Severity and Disease Activity of Emphysema, Airway Disease, and Interstitial Lung Disease

Goldin¹

2021

Respiration

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There has been an explosion of use for quantitative image analysis in the setting of lung disease due to advances in acquisition protocols and postprocessing technology, including machine and deep learning. Despite the plethora of published papers, it is important to understand which approach has clinical validation and can be used in clinical practice. This paper provides an introduction to quantitative image analysis techniques being used in the investigation of lung disease and focusses on the techniques that have a reasonable clinical validation for being used in clinical trials and patient care.

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“…Physiological parameters like the forced vital capacity (FVC) and transfer lung factor for carbon monoxide (TLCO) do not always represent the extent of fibrosis, and are nondiscriminating from other possible coexistent conditions, such as emphysema or pulmonary hypertension 4 . Using computed tomography (CT), several computer‐generated and visual scores have been reported 4–7 . Visual approaches, however, are inherently subjective.…”

mentioning

confidence: 99%

Quantification of MRI T2 Interstitial Lung Disease Signal‐Intensity Volume in Idiopathic Pulmonary Fibrosis: A Pilot Study

Benlala

Albat

Blanchard

et al. 2020

Magnetic Resonance Imaging

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Background Imaging has played a pivotal role in the diagnosis of idiopathic pulmonary fibrosis (IPF). Recent reports suggest that T2‐weighted MRI could be sensitive to monitor signal‐intensity modifications of the lung parenchyma, which may relate to the disease activity in IPF. However, there is a lack of automated tools to reproducibly quantify the extent of the disease, especially using MRI. Purpose To assess the feasibility of T2 interstitial lung disease signal‐intensity volume quantification using a semiautomated method in IPF. Study Type Single center, retrospective. Population A total of 21 adult IPF patients and four control subjects without lung interstitial abnormalities. Field Strength/Sequence Both free‐breathing ultrashort echo time (TE) lung MRI using the spiral volume interpolated breath hold examination (VIBE) sequence (3D‐UTE) and T2‐BLADE at 1.5T. Assessment Semiautomated segmentation of the lung volume was done using 3D‐UTE and registered to the T2‐BLADE images. The interstitial lung disease signal‐intensity volume (ISIV) was quantified using a Gaussian mixture model clustering and then normalized to the lung volume to calculate T2‐ISIV. The composite physiological index (CPI) and forced vital capacity (FVC) were measured as known biomarkers of IPF severity. Measurements were performed independently by three readers and averaged. The reproducibility between measurements was also assessed. Statistical Tests Reproducibility was assessed using the intraclass correlation coefficient (ICC) and Bland–Altman analysis. Correlations were assessed using Spearman test. Comparison of median was assessed using the Mann–Whitney test. Results The reproducibility of T2‐ISIV was high, with ICCs = 0.99. Using Bland–Altman analysis, the mean differences were found between –0.8 to 0.1. T2‐ISIV significantly correlated with CPI and FVC (rho = 0.48 and 0.50, respectively; P < 0.05). T2‐ISIV was significantly higher in IPF than in controls (P < 0.05). Data Conclusion T2‐ISIV appears to be able to reproducibly assess the volumetric extent of abnormal interstitial lung signal‐intensity modifications in patients with IPF, and correlate with disease severity. Level of Evidence 4 Technical Efficacy Stage 1

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

Cited by 8 publications

References 10 publications

Pulmonary vascular dysfunction without pulmonary hypertension: A distinct phenotype in idiopathic pulmonary fibrosis

Pulmonary vascular dysfunction without pulmonary hypertension: A distinct phenotype in idiopathic pulmonary fibrosis

The Emerging Role of Quantification of Imaging for Assessing the Severity and Disease Activity of Emphysema, Airway Disease, and Interstitial Lung Disease

Quantification of MRI T2 Interstitial Lung Disease Signal‐Intensity Volume in Idiopathic Pulmonary Fibrosis: A Pilot Study

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