The development of the lung involves a diverse group of molecules that regulate cellular
processes, organ formation, and maturation. The various stages of lung development are …

This retrospective, multicentre study evaluated patients with lymphangioleiomyomatosis
(LAM) and pre-capillary pulmonary hypertension (PH) by right heart catheterisation. It was …

Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease (ILD) of unknown
aetiology with a median survival of only 2–5 years. It is characterized by progressive …

Idiopathic pulmonary fibrosis (IPF) is a form of idiopathic interstitial pneumonia characterized
by temporally and spatially heterogeneous fibroblast proliferation and poor prognosis. No …

Background and objective Elevated mean pulmonary artery pressure (mPAP) is a significant
prognostic indicator in idiopathic pulmonary fibrosis (IPF). It has been reported that the …

Idiopathic pulmonary fibrosis (IPF) is a lethal, fibroproliferative disease. Pulmonary
hypertension (PH) can develop secondary to IPF and increase mortality. Alternatively …

Background Pirfenidone is a novel anti-fibrotic drug that has shown efficacy in five
randomized multicenter clinical trials enrolling patients with Idiopathic Pulmonary Fibrosis of …

Pulmonary hypertension is a relevant interceding morbidity in patients with pulmonary
fibrosis that has significant impact on exercise tolerance and outcome. The aim of this study …

Background The purpose of this study was to evaluate the prevalence and outcomes of
pulmonary hypertension in chronic hypersensitivity pneumonitis and to examine the …

Background: Pulmonary hypertension (PH) is a well-established complication in interstitial
lung disease (ILD) patients. The aim of this study is to investigate the physiological and …