Survival in patients with pulmonary hypertension associated with fibrotic interstitial lung disease (ILD) is independent of ILD subtype
Year: 2025
Type: article
Study design: Observational Study
Abstract: Extract Pulmonary hypertension (PH) is common in patients with interstitial lung disease (ILD-PH), observed in 7–15% of patients with early idiopathic pulmonary fibrosis (IPF) [1] and in over 70% in late stages of the disease [2]. PH drives an increase in mortality which appears independent of the hemodynamic severity in patients with ILD-PH [3]. Historic ILD registries suggest that survival is dependent upon ILD subtype—for instance, overall survival in non-IPF subtypes, including nonspecific interstitial pneumonia (NSIP), greatly exceeds that of IPF [4]. However, once PH intervenes, survival is considered driven by PH irrespective of the underlying ILD subtype. To date, this statement has been based on studies of individual cohorts [5, 6], rather than comparison between ILD-PH subtypes within centres. As such, we studied the survival of patients with fibrotic ILD and right heart catheterisation (RHC)-confirmed PH managed in a tertiary PH centre.
Source: ERJ Open Research
Authors: Ifan Jenkin, Konstantinos Dimopoulos, Steven D. Nathan, Sophie Herbert, Timothy Dawes, Simon Bax, Colm McCabe, Heba Nashat, Bhavin Rawal, Bhashkar Mukherjee, Vasilis Kouranos, Maria Kokosi, Athol U. Wells, Elisabetta Renzoni, Philip Molyneaux, Gisli Jenkins, Peter M. George, S. John Wort, Laura Claire Price
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $808
OpenAlex ID: W4415260191