Pulmonary hypertension in fibrosing idiopathic interstitial pneumonia: Uncertainties, challenges and opportunities

Girgis, Reda E.; Hoeper, Marius M.

doi:10.1016/j.healun.2021.03.004

Cited by 3 publications

(2 citation statements)

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“…Dyslipidaemia, as well as the existence of PH (medical records), echocardiographically measured right ventricular function (TAPSE), and PAPsys were of prognostic significance. In addition to male sex and age > 70 years, the degree of dyspnoea; DLCO < 60% pred., 6-MWD < 250 m, and SpO 2 < 88% measured during the 6-MWD; and the existence of PH and cardiovascular comorbidities were considered prognostically relevant baseline data in IPF patients [ 13 , 65 , 66 ]. Additionally, in the literature, histological data (number of fibroblastic foci), the extent of fibrotic alterations measured with HRCT, and selected biomarkers were prognostically relevant.…”

Section: Discussionmentioning

confidence: 99%

Value of Cardiopulmonary Exercise Testing in Prognostic Assessment of Patients with Interstitial Lung Diseases

Stubbe

Ittermann

Grieger

et al. 2022

JCM

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Background: Interstitial lung disease (ILD) is associated with high rates of comorbidities and non-infectious lung disease mortality. Against this background, we aimed to evaluate the prognostic capacity of lung function and cardiopulmonary exercise testing (CPET) in patients with ILD. Materials and Methods: A total of 183 patients with diverse ILD entities were included in this monocentric analysis. Prediction models were determined using Cox regression models with age, sex, body mass index (BMI), and all parameters from pulmonary function testing and CPET. Kaplan–Meier curves were plotted for selected variables. Results: The median follow-up period was 3.0 ± 2.5 years. Arterial hypertension (57%) and pulmonary hypertension (38%) were the leading comorbidities. The Charlson comorbidity index score was 2 ± 2 points. The 3-year and 5-year survival rates were 68% and 50%, respectively. VO2peak (mL/kg/min or %pred.) was identified as a significant prognostic parameter in patients with ILD. The cut-off value for discriminating mortality was 61%. Conclusion: The present analyses consistently revealed the high prognostic power of VO2peak %pred. and other parameters evaluating breathing efficacy (VÉ/VCO2 @AT und VÉ/VCO2 slope) in ILD patients. VO2peak %pred., in contrast to the established prognostic values FVC %pred., DLCO/KCO %pred., and GAP, showed an even higher prognostic ability in all statistical models.

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Section: Discussionmentioning

confidence: 99%

Value of Cardiopulmonary Exercise Testing in Prognostic Assessment of Patients with Interstitial Lung Diseases

Stubbe

Ittermann

Grieger

et al. 2022

JCM

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“…Interstitial lung disease (ILD) is a group of clinical disorders characterized by different degrees of inflammation and fibrosis in the lung interstitial tissues, such as idiopathic pulmonary fibrosis (IPF), and idiopathic interstitial pneumonia (IIP). The subtype and stage of ILD was the main influence factor and it was associated with IPF severity, 8–83% of patients with idiopathic pulmonary fibrosis (IPF) progressively develop PH, which resulted in lower exercise capacity, decreased quality of life, greater need for supplemental oxygen, and higher mortality compared to ILD alone ( 1 , 2 ). Precapillary pulmonary hypertension is defined as an elevation in mean pulmonary arterial pressure and pulmonary vascular resistance ( 3 ).…”

Section: Introductionmentioning

confidence: 99%

PAH-specific therapy for pulmonary hypertension and interstitial lung disease: A systemic review and meta-analysis

Zhao

Zhang

Zhou

et al. 2022

Front. Cardiovasc. Med.

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ObjectivePulmonary hypertension (PH) in context with interstitial lung disease (ILD) portends serious clinical consequences and a high rate of mortality. Recently published randomized controlled trials (RCTs) which assessed the pulmonary arterial hypertension (PAH)-specific drugs for pulmonary hypertension and interstitial lung disease (PH-ILD) revealed inconsistent clinical outcomes with previous studies. We conducted a systemic review and meta-analysis to further investigate the effect of PAH-specific therapies for PH-ILD.MethodsClinical trials were searched from the EMBASE, PUBMED, and CENTRAL databases. The duration from the establishment of the database to June 2022 for RCTs evaluates the effect of PAH-specific therapy in patients with PH-ILD. RevMan 5.4 was used for the meta-analysis.ResultsA total of six articles (with a total of 791 patients) were included, including 412 patients in the treated group and 379 patients in the control group. As compared to placebo, the change of 6MWD was a significant improvement with PAH-specific therapy in the six RCTs (23.09; 95% CI, 12.07–34.12 P < 0.0001); but when the study with inhaled treprostinil was excluded, the significant improvement in the change of 6MWD from baseline was not present anymore (MD 11.01, 95%CI−6.43–28.46 P = 0.22). There was no significant improvement in the change in lung function, hemodynamic parameters, clinical worsening, all-cause death, and serious adverse effects in the treated group compared to placebo.ConclusionPAH-specific therapy significantly improved exercise capacity in the patients with PH-ILD, but this is due to the greater contribution of the study with inhaled treprostinil. Therefore, our findings still did not support the routine use of the whole PAH-specific drugs for PH-ILD.

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Pulmonary Hypertension in Interstitial Lung Disease

Baldi

Souza

2022

Archivos de Bronconeumología

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Pulmonary hypertension in fibrosing idiopathic interstitial pneumonia: Uncertainties, challenges and opportunities

Cited by 3 publications

References 100 publications

Value of Cardiopulmonary Exercise Testing in Prognostic Assessment of Patients with Interstitial Lung Diseases

Value of Cardiopulmonary Exercise Testing in Prognostic Assessment of Patients with Interstitial Lung Diseases

PAH-specific therapy for pulmonary hypertension and interstitial lung disease: A systemic review and meta-analysis

Pulmonary Hypertension in Interstitial Lung Disease

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