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Updated: Nov 29, 2025

Establishment and Validation of a Rat Model of Pulmonary Arterial Hypertension Associated with Pulmonary Fibrosis
Published on: May 23, 2025
Pulmonary hypertension secondary to pulmonary fibrosis: clinical data, histopathology and molecular insights
Grégoire Ruffenach1, Jason Hong1,2, Mylène Vaillancourt3
1Division of Molecular Medicine, Department of Anesthesiology and Perioperiative Medicine, David Geffen School of Medicine, University of California, BH-550CHS, Los Angeles, CA, 90095-7115, USA.
Abstract:
Pulmonary hypertension (PH) developing secondarily in pulmonary fibrosis (PF) patients (PF-PH) is a frequent co-morbidity. The high prevalence of PH in PF patients is very concerning since the presence of PH is a strong predictor of mortality in PF patients. Until recently, PH was thought to arise solely from fibrotic destruction of the lung parenchyma, leading to hypoxic vasoconstriction and loss of vascular bed density. Thus, potential cellular and molecular dysregulation of vascular remodeling as a driver of PF-PH has been under-investigated. The recent demonstrations that there is no correlation between the severity of the fibrosis and development of PH, along with the finding that significant vascular histological and molecular differences exist between patients with and without PH have shifted the etiological paradigm of PF-PH. This review aims to provide a comprehensive translational overview of PH in PF patients from clinical diagnosis and outcome to the latest understanding of the histology and molecular pathophysiology of PF-PH.
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