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Diffuse Lung Disease |

Pulmonary Hypertension in Idiopathic Pulmonary Fibrosis: A New Phenotype?

Margarida Redondo, MD; Diogo Costa, PhD; Natalia Melo, MD; Patricia Mota, MD; Antonio Morais, MD
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Centro Hospitalar de São João, Porto, Portugal


Chest. 2014;145(3_MeetingAbstracts):240A. doi:10.1378/chest.1825838
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Abstract

SESSION TITLE: ILD Posters

SESSION TYPE: Poster Presentations

PRESENTED ON: Saturday, March 22, 2014 at 01:15 PM - 02:15 PM

PURPOSE: Idiopathic Pulmonary Fibrosis (IPF) is a progressive fatal diffuse parenchymal disease. Pulmonary hypertension (PH) in IPF has been increasingly recognized as a condition with significant prognostic relevance. PH usually develops in patients with advanced IPF however there exists a subset of patients who develop PH at earlier stages. The objective of this study is to compare clinical, functional and radiological parameters in patients with IPF with and without PH at presentation.

METHODS: Fifty-three patients with IPF diagnosis according to the criteria of the ATS/ERS were included. Based on echcardiography parameters, PH was defined as a systolic pulmonary artery pressure above 35 mmHg. Patients with left heart disease were excluded.

RESULTS: From fifty-three patients included, 60.4% were males, with a mean age at diagnosis of 69.2 years-old (sd=10.62). PH was present in 20.8% of patients at diagnosis. There were no statistically significant differences between the two groups of patients regarding to the time elapsed from onset of symptoms to diagnosis and to the presence of emphysema. Patients with PH had a lower mean diffusing capacity of the lung for carbon monoxide (p=0.035) and a lower PaO2 (p=0.049); no significant differences regarding other functional parameters were found, including FVC and TLC. There were also no statistically significant differences between the two groups of patients regarding to the score of fibrosis.

CONCLUSIONS: In this study, the presence of PH at diagnosis did not correlate with the usual parameters that characterize disease severity, such as lung volumes and fibrosis score on HRCT. These data are consistent with the concept that IPF and PH in the earlier stages of the disease correspond to a distinct phenotype that is likely to result from different pathophysiological mechanisms.

CLINICAL IMPLICATIONS: In future this concept may generate therapeutic approaches.

DISCLOSURE: The following authors have nothing to disclose: Margarida Redondo, Diogo Costa, Natalia Melo, Patricia Mota, Antonio Morais

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