Exploration of Metabolome in Patients With Interstitial Lung Disease and Pulmonary Hypertension With or Without Specific Pulmonary Hypertension Treatment

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Conditions studied: Interstitial Lung Disease, Precapillar Pulmonary Hypertension

In brief

Fibrosing interstitial lung diseases (FILDs) encompass a group of rare diseases characterized by progressive pulmonary fibrosis leading to respiratory failure. Current treatments primarily aim to slow disease progression but remain limited, making lung transplantation the ultimate recourse. The development of pulmonary hypertension (PH) in the context of FILDs significantly worsens morbidity and mortality and drastically reduces patients' life expectancy. Conventional treatments for PH are generally ineffective in this setting. Nevertheless, some promising therapeutic agents are currently under investigation, particularly inhaled prostacyclin analogs such as treprostinil, which have demonstrated efficacy in recent clinical studies. Our study aims to explore, in a minimally invasive manner, variations in metabolites in the serum and urine of patients with PH secondary to FILDs, before and during treatment. The main objective is to better understand the systemic effect of these treatments. Furthermore, the identification of metabolomic signatures will allow us to differentiate responders from non-responders, thus providing valuable prognostic and predictive criteria. To date, some patients do not benefit from the available treatments, and better selection of responders could prevent iatrogenic effects in patients whose clinical condition is already fragile. In addition, characterizing the systemic mode of action of these treatments could pave the way for new clinical research focused on the profiles of responding patients. Finally, a thorough understanding of the efficacy of the studied therapies is essential. Indeed, effective treatment of PH in the context of FILDs could not only slow disease progression but also reduce the need for lung transplantation, a major challenge in a context of organ shortage.

Key facts

Study ID
NCT07254338
Run by
Hospices Civils de Lyon
People needed
80
Starts
2026-02-16
Expected to finish
2029-08-16
Last updated by the study team
2026-02-17

Who can join

Age: 18 and older. Sex: any. Healthy volunteers: accepted.

You may qualify if…

You may not qualify if…

Where it is running

Full record on ClinicalTrials.gov

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