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Diaphragmatic ultrasound findings correlate with dyspnea, exercise tolerance, health-related quality of life and lung function in patients with fibrotic interstitial lung disease.

ABSTRACT: BACKGROUND:Fibrotic interstitial lung disease (FILD) patients are typically dyspneic and exercise-intolerant with consequent impairment of health-related quality of life (HRQoL). Respiratory muscle dysfunction is among the underlying mechanisms of dyspnea and exercise intolerance in FILD but may be difficult to diagnose. Using ultrasound, we compared diaphragmatic mobility and thickening in FILD cases and healthy controls and correlated these findings with dyspnea, exercise tolerance, HRQoL and lung function. METHODS:We measured diaphragmatic mobility and thickness during quiet (QB) and deep breathing (DB) and calculated thickening fraction (TF) in 30 FILD cases and 30 healthy controls. We correlated FILD cases' diaphragmatic findings with dyspnea, exercise tolerance (six-minute walk test), lung function and HRQoL (St. George's Respiratory Questionnaire). RESULTS:Diaphragmatic mobility was similar between groups during QB but was lower in FILD cases during DB when compared to healthy controls (3.99?cm vs 7.02?cm; p

SUBMITTER: Santana PV

PROVIDER: S-EPMC6802109 | biostudies-literature | 2019 Oct

REPOSITORIES: biostudies-literature

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Diaphragmatic ultrasound findings correlate with dyspnea, exercise tolerance, health-related quality of life and lung function in patients with fibrotic interstitial lung disease.

Santana Pauliane Vieira PV Cardenas Leticia Zumpano LZ de Albuquerque André Luis Pereira ALP de Carvalho Carlos Roberto Ribeiro CRR Caruso Pedro P

BMC pulmonary medicine 20191021 1

<h4>Background</h4>Fibrotic interstitial lung disease (FILD) patients are typically dyspneic and exercise-intolerant with consequent impairment of health-related quality of life (HRQoL). Respiratory muscle dysfunction is among the underlying mechanisms of dyspnea and exercise intolerance in FILD but may be difficult to diagnose. Using ultrasound, we compared diaphragmatic mobility and thickening in FILD cases and healthy controls and correlated these findings with dyspnea, exercise tolerance, HR ...[more]

PMID: 31638951

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Project description:BackgroundDehydroepiandrosterone (DHEA) is a precursor sex hormone with antifibrotic properties. The aims of this study were to investigate antifibrotic mechanisms of DHEA, and to determine the relationship between DHEA-sulfate (DHEAS) plasma levels, disease severity and survival in patients with fibrotic interstitial lung diseases (ILDs).MethodsHuman precision cut lung slices (PCLS) and normal human lung fibroblasts were treated with DHEA and/or transforming growth factor (TGF)-β1 before analysis of pro-fibrotic genes and signal proteins. Cell proliferation, cytotoxicity, cell cycle and glucose-6-phosphate dehydrogenase (G6PD) activity were assessed. DHEAS plasma levels were correlated with pulmonary function, the composite physiologic index (CPI), and time to death or lung transplantation in a derivation cohort of 31 men with idiopathic pulmonary fibrosis (IPF) and in an independent validation cohort of 238 men and women with fibrotic ILDs.ResultsDHEA decreased the expression of pro-fibrotic markers in-vitro and ex-vivo. There was no cytotoxic effect for the applied concentrations, but DHEA interfered in proliferation by modulating the cell cycle through reduction of G6PD activity. In men with IPF (derivation cohort) DHEAS plasma levels in the lowest quartile were associated with poor lung function and higher CPI (adjusted OR 1.15 [95% CI 1.03-1.38], p = 0.04), which was confirmed in the fibrotic ILD validation cohort (adjusted OR 1.03 [95% CI 1.00-1.06], p = 0.01). In both cohorts the risk of early mortality was higher in patients with low DHEAS levels, after accounting for potential confounding by age in men with IPF (HR 3.84, 95% CI 1.25-11.7, p = 0.02), and for age, sex, IPF diagnosis and prednisone treatment in men and women with fibrotic ILDs (HR 3.17, 95% CI 1.35-7.44, p = 0.008).ConclusionsDHEA reduces lung fibrosis and cell proliferation by inducing cell cycle arrest and inhibition of G6PD activity. The association between low DHEAS levels and disease severity suggests a potential prognostic and therapeutic role of DHEAS in fibrotic ILD.

Dataset Information

Diaphragmatic ultrasound findings correlate with dyspnea, exercise tolerance, health-related quality of life and lung function in patients with fibrotic interstitial lung disease.

Publications

Diaphragmatic ultrasound findings correlate with dyspnea, exercise tolerance, health-related quality of life and lung function in patients with fibrotic interstitial lung disease.

Similar Datasets

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