Idiopathic pulmonary fibrosis and related lung-scarring diseases progress despite the two approved antifibrotic drugs, pirfenidone and nintedanib, which only slow the decline. Several new drug classes are now in late-stage testing, offering the first real chance of meaningfully better treatment in over a decade.
What's actually going on in research
Trials are testing PDE4B inhibitors, LPA receptor blockers, and other antifibrotic drugs, often in combination with existing therapies. Researchers are also studying progressive forms of other interstitial lung diseases, lung transplant outcomes, treatments for cough and shortness of breath, and genetic testing to identify familial cases.
New antifibrotics
Drugs like nerandomilast (a PDE4B inhibitor) showed slowed lung-function decline in trials and could become the third approved antifibrotic. Several other drug classes are also advancing.
Combination therapy
Studies are testing whether adding new antifibrotics to pirfenidone or nintedanib slows disease more than either drug alone, with manageable side effects.
Beyond IPF
Many other interstitial lung diseases progress similarly to IPF. Trials are testing antifibrotics across these conditions, expanding who can be treated.
What to know before you search
Eligibility often depends on type of fibrosis (IPF or other), lung function tests, recent decline rate, oxygen needs, and other lung conditions.
What types of trials are currently open
- New medication trials — Testing antifibrotic drugs and other treatments aimed at slowing lung scarring.
- Therapy strategy trials — Testing combinations of antifibrotic drugs to slow decline more than one drug alone.
- Surgical trials — Studies of lung transplant approaches and outcomes.
- Symptom trials — Testing treatments for chronic cough, breathlessness, and oxygen therapy.
- Observational studies — Following people with pulmonary fibrosis to understand progression and identify predictors.
Recently added Pulmonary Fibrosis trials
Advanced Imaging to Assess the Effect of Immunosuppression on Progressive Fibrosis
The purpose of this study is to investigate how immunosuppression treatment affects measurements of active collagen deposition using \[68Ga\]CBP8 positron emission tomography (PET) and tissue injury using dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) in individuals with non-idiopathic pulmonary fibrosis interstitial lung disease (non-IPF ILD).
Proof of Principle Study for an Efficacy Trial of Linaclotide for Cystic Fibrosis
Linaclotide is a medicine used to treat constipation and irritable bowel syndrome with constipation (IBS-C). It works by acting on the surface of the gut lining, where it increases the movement of salt and water into the bowel. This softens stools, makes them easier to pass, and can also reduce gut pain One advantage of linaclotide is that, unlike some natural substances in the gut, it is stable and can act throughout the intestine. Studies in animals show that it has the strongest effect in the upper small intestine, but it may act in other parts of the bowel as well. In people, however, it is not yet clear whether linaclotide mainly works in the small intestine or in the large intestine (colon). Knowing this is important, because it could help the investigators understand whether linaclotide might also be useful in other conditions, such as cystic fibrosis, where the gut does not handle fluid properly. Linaclotide is taken as a capsule, but less than 1% is absorbed into the bloodstream. Instead, it stays in the gut, where it is broken down into smaller active parts. This means both the small intestine and colon may be exposed to its effects. Until now, it has been hard to study this because traditional methods only measure one part of the gut at a time. A team at the University of Nottingham has developed MRI scanning methods that can safely and non-invasively measure water content in the small intestine and colon. The aim of this pilot study is to use MRI in healthy volunteers to see exactly where linaclotide acts. This knowledge will help optimise future studies in conditions such as cystic fibrosis.
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