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March 20, 2023
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Q&A: Treating pulmonary hypertension-ILD with high-dose inhaled treprostinil

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Inhaled treprostinil is used to treat pulmonary hypertension with interstitial lung disease, and a study recently found that higher doses led to better clinical outcomes than a lower dose or placebo.

In a post-hoc analysis of the INCREASE study published in CHEST, researchers analyzed 326 patients with pulmonary hypertension-associated interstitial lung disease (PH-ILD) to determine if receiving higher doses of inhaled treprostinil (Tyvaso, United Therapeutics) delayed clinical worsening and yielded clinical improvement over 16 weeks.

Quote from Steven D. Nathan, MD

As Healio previously reported, researchers found higher incidences of clinical worsening in the placebo and low dose groups (< 9 breaths per session) compared with the high-dose treprostinil group (≥ 9 breaths per session) between week 4 and week 16 (33.7% vs. 22.8% vs. 17.1%, respectively; P = .006).

In terms of clinical improvement at the end of the study period, more patients on high and low dose treprostinil demonstrated improvement than patients receiving placebo (15.7% vs. 12.7% vs. 4.6%, respectively; P = .003).

To learn more about inhaled treprostinil treatment and these findings, Healio spoke with lead study researcher Steven D. Nathan, MD, director of the advanced lung disease program and the lung transplant program at Inova Fairfax Hospital in Falls Church, Virginia.

Healio: How prevalent is PH-ILD?

Nathan: The prevalence of ILD is maybe around 500,000 to 600,000 in the U.S., and the prevalence of pulmonary hypertension ranges from 10% to 50% among these patients.

Healio: Were any of the study findings unexpected or surprising?

Nathan: Yes, in this study we found that the number of patients needed to treat with inhaled treprostinil to prevent one clinical worsening episode or cause one clinical improvement episode was very small at just four patients.

Healio: Would you briefly characterize what the impact of the findings are for the everyday clinician?

Nathan: These findings lend more support to the use of inhaled treprostinil in patients with PH-ILD. Not only does it prevent worsening, but it can also result in improvement which is something that is not typically seen in fibrotic lung disease. This should heighten clinicians’ awareness of the importance of routinely screening patients for PH and ILD. If there is any suspicion of PH, then a right heart catheterization is always needed to confirm the diagnosis.

Healio: How will future studies be different?

Nathan: The results of this study has led to an increasing interest in studying other agents for this indication, namely PH-ILD.

Healio: What should readers remember walking away from your study?

Nathan: Readers should remember that inhaled treprostinil has potentially multiple benefits for a group of patients with a very poor prognosis and not much else in the way of therapeutic options.

For more information:

Steven D. Nathan, MD, can be reached at steven.nathan@inova.org.

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