People with interstitial lung disease (ILD), such as idiopathic pulmonary fibrosis (IPF), can
sometimes also develop pulmonary hypertension – high blood pressure in the lungs.
Unfortunately, there are no approved medicines for pulmonary hypertension in interstitial lung disease, and survival can be poor, especially in severe cases.
A large international study titled “Hemodynamics and Phosphodiesterase-5 Inhibitor Treatment Associated with Survival in ILD-PH: a PVRI GoDeep Meta-Registry Analysis” analysed real-world data from almost 1,000 people with interstitial lung disease-pulmonary hypertension. It found that:
- Many patients were treated with phosphodiesterase-5 inhibitors (PDE5i) – medicines already used for some other types of pulmonary hypertension (for example: tadalafil, sildenafil)
- In severe idiopathic pulmonary fibrosis-pulmonary hypertension (PVR > 5), taking PDE5i was linked to significantly better survival, especially in idiopathic pulmonary fibrosis and other idiopathic forms of lung fibrosis.
- The best results were seen when PDE5i was combined with an inhaled prostacyclin analogue (another pulmonary hypertension medicine).
What this means for patients:
If you have severe pulmonary hypertension with interstitial lung disease, especially idiopathic pulmonary fibrosis, treatment with PDE5 inhibitors – and possibly in combination with other therapies – might improve survival. More research is needed to confirm this, but these results are promising and could help guide future clinical trials.
Read more at this link on the National Library of Medicine
Summary by Louise Bouman, AfPH volunteer
Citation
Yogeswaran A, Hassoun PM, Saleh K, Fünderich M, Balasubramanian A, Konswa Z, Kiely DG, Lawrie A, Thenappan T, Eichstaedt CA, Grünig E, Wilkins MR, Howard L, Olschewski H, Kovacs G, Cajigas HR, Frantz R, Sabbour H, Sweatt AJ, Zamanian RT, Arvanitaki A, Giannakoulas G, Elwing J, Jose A, Beckmann S, Olsson KM, Stadler S, Held M, Halank M, Ewert R, Behr J, Milger-Kneidinger K, Pausch C, Pittrow D, Majeed RW, Wilhelm J, Ghofrani HA, Grimminger F, Tello K, Hoeper MM, Seeger W; PVRI-GoDeep-Consortium. Hemodynamics and Phosphodiesterase-5 Inhibitor Treatment Associated with Survival in ILD-PH: A PVRI GoDeep Meta-Registry Analysis. Am J Respir Crit Care Med. 2025 Aug 7. doi: 10.1164/rccm.202412-2371OC. Epub ahead of print. PMID: 40772955.
