Yutrepia use increases amid plans for new PAH, PH-ILD clinical trials

Yutrepia, an inhaled dry powder formulation of treprostinil that won regulatory approval in the U.S. in May 2025, was prescribed to more than 2,800 patients through December of last year, according to its developer Liquidia.

Overall, more than 2,200 patients have started treatment, per the biopharmaceutical company, which also noted that the number of prescribers increased to 750 over the same period.

The medication, delivered via an easy-to-use, low-effort inhalation device, is approved in the U.S. to improve exercise capacity in people with pulmonary arterial hypertension (PAH) and pulmonary hypertension associated with interstitial lung disease, known as PH-ILD.

“2025 marked a transformational year for Liquidia, with the successful commercial launch of Yutrepia,” Roger Jeffs, PhD, Liquidia’s CEO, said in a company press release announcing preliminary full-year net sales of the medication. Treprostinil, the active ingredient in Yutrepia, is a lab-made version of prostacyclin, a naturally occurring hormone that causes blood vessels to relax and widen, thus reducing blood pressure in people with PAH and PH-ILD.

“We are encouraged by the continued momentum in adoption, which reflects strong execution by our team and growing physician confidence in Yutrepia as a differentiated and increasingly preferred inhaled prostacyclin option,” Jeffs said.

This year, the company expects to complete ASCENT (NCT06129240), a U.S. study evaluating Yutrepia’s safety and tolerability over one year in people with PH-ILD. Early results indicated that Yutrepia improved exercise capacity in adults with the condition.

Also part of the company’s plans for this year is the launch of three open-label, multicenter studies to assess tolerability, dosing adjustments, and exploratory efficacy of Yutrepia treatment.

One study will involve PAH patients with an inadequate response to orally administered Uptravi (selexipag, sold by Johnson & Johnson), and other medicines mimicking prostacyclin. A second expects to enroll participants with PH-ILD who had an inadequate response to United Therapeutics‘ Tyvaso (inhaled treprostinil) or Tyvaso DPI (a dry powder formulation inhaled through a portable inhalation device). The third will enroll individuals with idiopathic pulmonary fibrosis — when the condition has no known cause — or progressive pulmonary fibrosis who have not been treated with inhaled treprostinil medicines.

In an investigator-initiated study, the company also plans to explore whether combining Yutrepia and Merck‘s Winrevair (sotatercept) helps reduce the need for injectable prostacyclin therapy.

Liquidia also advancing L606 into clinical testing

In addition to further clinical testing of Yutrepia, Liquidia expects to launch a new trial evaluating L606, a sustained-release formulation of inhaled treprostinil that it’s developing in partnership with Pharmosa Biopharm. L606 comprises treprostinil encapsulated within liposomes, or tiny fat-like particles, to enable more sustained drug exposure to the lungs and less frequent dosing than other dry powder formulations.

The Phase 3 Re-Spire trial (NCT07285655) will assess the safety and efficacy of L606 in more than 300 people with PH-ILD. Its primary goal is to determine if the medication increases patients’ exercise ability for up to six months. Participants will be randomly assigned to receive L606 or a placebo.

According to the company, results from this study are expected to support applications seeking the treatment’s regulatory approval.

A previous Phase 1 study (NCT04041648) in healthy adults showed therapeutic levels of L606 in blood for up to 12 hours. Another open-label Phase 3 study (NCT04691154) is evaluating the safety and tolerability of L606 in adults with PAH or PH-ILD.

Liquidia also planned to share updates at the 44th Annual J.P. Morgan Healthcare Conference, taking place from Jan. 12 through today in San Francisco.

PAH and PH-ILD are forms of pulmonary hypertension, which is characterized by high blood pressure, or hypertension, in the pulmonary arteries, the blood vessels that supply the lungs. In PAH, the narrowing of pulmonary arteries restricts blood flow through the lungs, while PH-ILD is associated with lung inflammation and scarring.

“Looking ahead to 2026, we are excited to build on this foundation by advancing clinical programs to further differentiate Yutrepia and L606, our extended-release treprostinil formulation, across current and potential future indications … where there remains a significant unmet need,” Jeffs said.