New therapies in development may improve treatment options for progressive pulmonary fibrosis patients.
Progressive pulmonary fibrosis (PPF), a serious form of interstitial lung disease, remains one of the most difficult lung conditions to treat.
Unlike idiopathic pulmonary fibrosis (IPF), PPF refers to a group of non-IPF lung diseases that continue to worsen over time even when patients are receiving appropriate care. As the disease progresses, it can cause irreversible scarring in the lungs, reduce breathing capacity, and significantly shorten survival.()
Current Antifibrotic Treatments Fall Short — But Pipeline Therapies Offer New Hope
Although currently available antifibrotic treatments can slow the loss of lung function, they do not stop the disease entirely—or reverse the damage that has already occurred. That leaves many patients facing continued decline, worsening symptoms, and a reduced quality of life. Now, according to healthcare analysts at GlobalData, a wave of late-stage therapies in development could mark an important shift in how PPF is treated.
At present, treatment options for PPF are largely adapted from therapies already used for IPF. These include Boehringer Ingelheim’s Ofev (nintedanib) and Jascayd (nerandomilast) in the United States. In some cases, doctors may also prescribe pirfenidone, though its use in PPF is generally considered off-label.
These drugs are often used alongside background immunomodulatory therapies tailored to the patient’s underlying interstitial lung disease. While this combination can help manage progression, experts say it still falls short of what many patients need—particularly when it comes to long-term survival, symptom relief, and preserving day-to-day functioning.
Admilparant, Tyvaso, and TPIP Emerge as Front-Runners in PPF Treatment Pipeline
Several investigational therapies are now drawing attention in the late-stage development pipeline, including Bristol Myers Squibb’s admilparant, United Therapeutics’ Tyvaso (treprostinil), and Insmed’s treprostinil palmitil (TPIP) .
Together, these candidates suggest the field may be moving beyond broad antifibrotic therapy toward more targeted and potentially more flexible treatment strategies.
Among the emerging candidates, admilparant is being watched closely for its potential to slow the build-up of scar tissue in the lungs more effectively than currently available options.
The therapy works by blocking lysophosphatidic acid receptor 1, a pathway involved in fibrosis. By interfering with fibroblast activity and reducing damage to lung cells, the drug is designed to help limit the processes that drive scarring.
Admilparant Could Emerge as a Leading PPF Treatment
In a Phase II study, admilparant was associated with a 69% lower rate of forced vital capacity (FVC) decline compared with placebo—a notable result in a disease where preserving lung function is critical.
Still, important questions remain. Researchers have not yet shown whether the drug can improve outcomes such as survival, hospitalizations, or flare-ups of the disease over the long term. Those answers may come from the upcoming Phase III trial.
Connor Daniels, Healthcare Analyst at GlobalData, said that if Bristol Myers Squibb can confirm both stronger efficacy and better tolerability in larger studies, admilparant could emerge as a leading option in the PPF treatment space.
Another therapy being explored for PPF is Tyvaso, an inhaled treatment already approved for pulmonary arterial hypertension and pulmonary hypertension associated with interstitial lung disease.
Could Tyvaso Help Curb Fibrosis in the Lungs?
Tyvaso works as a prostacyclin agonist and is believed to have anti-inflammatory and antifibrotic effects in the lungs. Early evidence suggests it may help reduce abnormal cell growth and collagen production—two key drivers of fibrosis.
The drug is now being tested in a Phase III study known as TETON-PPF, where it is being evaluated in combination with existing standard treatments.
While Tyvaso has not yet generated PPF-specific efficacy data, analysts say the ongoing trial could position it as a meaningful option earlier in the treatment journey if results are favorable.
Insmed’s treprostinil palmitil (TPIP) is also part of the next wave of inhaled therapies, but with a design intended to improve how the drug is delivered and tolerated.
Like Tyvaso, TPIP is based on treprostinil, but it is formulated as a prodrug that stays in lung tissue longer before gradually releasing the active medicine. This extended-release approach may allow for once-daily dosing, compared with the four-times-daily schedule often required with Tyvaso.
That difference could matter for both convenience and side effects. High peak concentrations with inhaled treprostinil can sometimes trigger issues such as coughing, and Insmed is expected to test whether TPIP can offer a smoother and better-tolerated experience for patients.
The company announced its Phase III plans in January 2026, though the trial has not yet begun.
Taken together, the latest pipeline developments suggest that the treatment landscape for progressive pulmonary fibrosis may be entering a new phase.
Rather than relying only on broad antifibrotic drugs, future care may increasingly involve targeted therapies, inhaled options, and combination treatment strategies designed to address different aspects of the disease.
For patients living with a condition that can steadily rob them of lung function and quality of life, that shift could eventually mean more personalized—and potentially more effective—ways to manage the disease.
As Daniels noted, the emerging pipeline reflects a broader transition in the PPF market: one that could give patients more treatment choices than ever before.
References:
- Late-stage pipeline to transform progressive pulmonary fibrosis treatment landscape, says GlobalData – (https://www.globaldata.com/media/pharma/late-stage-pipeline-to-transform-progressive-pulmonary-fibrosis-treatment-landscape-says-globaldata/)
Source-Medindia