The Syndrome of Combined Pulmonary Fibrosis and Emphysema

Cottin, Vincent; Cordier, Jean-François

doi:10.1007/978-1-4471-2401-6_22

Cited by 13 publications

(18 citation statements)

References 155 publications

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“…In fact, the threshold of 10% decline in FVC is below the degree of normal intraindividual variability, as suggested by recent guidelines [15]. Secondly, physiological measures provide only indirect estimates of the progression of fibrosis, and may be affected by coexisting emphysema and/or pulmonary hypertension that are frequently associated with IPF [7,8]. Lastly, PFTs may not be sensitive enough to detect subclinical progression of fibrosis: FVC decline as defined by current thresholds is a relatively rare event in IPF clinical trials, which has led some authors to suggest that marginal declines in FVC may be more sensitive, though less specific [16].…”

Section: Discussionmentioning

confidence: 99%

“…decline in forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (DLCO) over 6-12 months [2,5,6]. However, the utility of such longitudinal physiological indices for individual patient management is still significantly limited by substantial intra-individual variability, perhaps with confounding by coexisting conditions, such as emphysema or pulmonary hypertension [7,8].…”

Section: Introductionmentioning

confidence: 99%

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Automated quantification of radiological patterns predicts survival in idiopathic pulmonary fibrosis

Maldonado

Moua

Rajagopalan³

et al. 2013

European Respiratory Journal

201

139

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Accurate assessment of prognosis in idiopathic pulmonary fibrosis remains elusive due to significant individual radiological and physiological variability. We hypothesised that short-term radiological changes may be predictive of survival.We explored the use of CALIPER (Computer-Aided Lung Informatics for Pathology Evaluation and Rating), a novel software tool developed by the Biomedical Imaging Resource Laboratory at the Mayo Clinic Rochester (Rochester, MN, USA) for the analysis and quantification of parenchymal lung abnormalities on high-resolution computed tomography. We assessed baseline and follow-up (time-points 1 and 2, respectively) high-resolution computed tomography scans in 55 selected idiopathic pulmonary fibrosis patients and correlated CALIPER-quantified measurements with expert radiologists' assessments and clinical outcomes.Findings of interval change (mean 289 days) in volume of reticular densities (hazard ratio 1.91, p50.006), total volume of interstitial abnormalities (hazard ratio 1.70, p50.003) and per cent total interstitial abnormalities (hazard ratio 1.52, p50.017) as quantified by CALIPER were predictive of survival after a median follow-up of 2.4 years. Radiologist interpretation of short-term global interstitial lung disease progression, but not specific radiological features, was also predictive of mortality.These data demonstrate the feasibility of quantifying interval short-term changes on high-resolution computed tomography and their possible use as independent predictors of survival in idiopathic pulmonary fibrosis. @ERSpublications Short-term quantified CT changes are predictive of survival in IPF

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Automated quantification of radiological patterns predicts survival in idiopathic pulmonary fibrosis

Maldonado

Moua

Rajagopalan³

et al. 2013

European Respiratory Journal

201

139

View full text Add to dashboard Cite

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“…The syndrome of CPFE results from the association of distinct features and symptoms, that include severe dyspnoea, unexpected subnormal spirometry findings, severely impaired transfer capacity for carbon monoxide, hypoxaemia at exercise, and characteristic imaging features [8,[13][14][15]. Basal crackles are heard on auscultation (table 1) [4,9].…”

Section: Disease Characteristicsmentioning

confidence: 99%

“…Conversely, it has also been suggested that the presence of pulmonary emphysema modifies the outcome of patients with IPF [5]. Thus, CPFE deserves to be termed a ''syndrome'' because of the association of symptoms and clinical manifestations, each with a probability of being present increased by the presence of the others [8]. CPFE also warrants inclusion as a distinct pulmonary manifestation within the spectrum of connective tissue disease-associated lung diseases, such as rheumatoid arthritis and systemic sclerosis [19].…”

Section: Disease Characteristicsmentioning

confidence: 99%

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The impact of emphysema in pulmonary fibrosis

Cottin¹

2013

European Respiratory Review

131

115

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Several groups have described a syndrome in which idiopathic pulmonary fibrosis (IPF) coexists with pulmonary emphysema. This comes as no surprise since both diseases are associated with a history of exposure to cigarette smoke. The syndrome of combined pulmonary fibrosis and emphysema (CPFE) is characterised by upper lobe emphysema and lower lobe fibrosis. Physiological testing of these patients reveals preserved lung volume indices contrasted by markedly impaired diffusion capacity. The incidence of CPFE remains unknown but several case series suggest that this subgroup may comprise up to 35% of patients with IPF. CPFE is a strong determinant of associated pulmonary hypertension (PH). In addition, CPFE has major effects on measures of physiological function, exercise capacity and prognosis, and may affect the results of pulmonary fibrosis trials. Further studies are needed to ascertain the aetiology, morbidity, mortality and management of the CPFE syndrome, with or without PH, and to evaluate novel therapeutic options in CPFE.

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The Effects of Pulmonary Vasodilating Agents on Right Ventricular Parameters in Severe Group 3 Pulmonary Hypertension: A Pilot Study

et al. 2016

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Pulmonary arterial hypertension (PAH)-approved vasodilators improve right ventricular (RV) function in patients with PAH. However, whether PAH-approved drugs ameliorate RV morphology and function in lung disease-associated pulmonary hypertension (lung-PH) remains unclear. We aimed to prospectively evaluate the changes in RV volume and ejection fraction (RVEF) in 14 consecutive severe lung-PH patients treated with PAH-approved vasodilators. Severe lung-PH was defined as a mean pulmonary arterial pressure (MPAP) of ≥35 mmHg or an MPAP of ≥25 mmHg with a cardiac index (L/min/m 2 ) of <2. Right heart catheterization and cardiac magnetic resonance (CMR) imaging were performed at baseline and at 3 months after starting sildenafil with or without other PAH-approved drugs. Follow-up was conducted at 3 months in 11 participants; compared with baseline values, MPAP and pulmonary vascular resistance (PVR) decreased by 18% and 37%, respectively. Baseline CMR imaging revealed an elevated RV end-diastolic volume index (RVEDVI; mL/m 2 ) of 117.5 ± 35.9 and a below-average RVEF of 25.2% ± 7.2%; after 3 months, RVEDVI decreased by 23.7% (P = 0.0061) and RVEF increased by 32.9% (P = 0.0165). Among the 11 patients, 3 were thought to be a stable and homogenous subset in terms of background lung disease and medical management administered. These 3 patients exhibited similar ameliorations in PVR and RVEF, compared with the other 8 patients. PAH-approved drug treatment may improve RV dilatation and systolic function among patients with severe lung-PH. This study was approved by University Hospital Medical Information Network Clinical Trials Registry (UMIN-CTR) on September 1, 2013 (UMIN000011541).

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The Syndrome of Combined Pulmonary Fibrosis and Emphysema

Cited by 13 publications

References 155 publications

Automated quantification of radiological patterns predicts survival in idiopathic pulmonary fibrosis

Automated quantification of radiological patterns predicts survival in idiopathic pulmonary fibrosis

The impact of emphysema in pulmonary fibrosis

The Effects of Pulmonary Vasodilating Agents on Right Ventricular Parameters in Severe Group 3 Pulmonary Hypertension: A Pilot Study

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