Idiopathic pulmonary fibrosis causes scarring in the lungs that gradually makes breathing harder. Around 100,000 people in the U.S. live with IPF, diagnosed most often after age 60. Two approved drugs—pirfenidone and nintedanib—slow progression but don't reverse scarring, leaving median survival around three to five years from diagnosis.
What's actually going on in research
Trials are testing drugs that target different scarring pathways, inhaled therapies that reach lung tissue directly, combination treatments pairing the two approved drugs, and biologics that dampen specific immune signals. Researchers are also studying biomarkers to predict who will progress quickly and testing drugs that worked in other fibrotic diseases.
Anti-fibrotic combinations
Studies are testing whether using pirfenidone and nintedanib together slows scarring more than either drug alone. Early data suggest the combination may be tolerable for some patients.
Inhaled therapies
New drugs delivered by inhaler aim to reach scarred lung tissue at higher concentrations while reducing side effects. Several are in mid-stage trials.
Autotaxin inhibitors
These drugs block an enzyme involved in lung scarring. One candidate failed in recent trials, but researchers are testing whether different timing or patient selection might work better.
What to know before you search
Eligibility typically depends on lung function test results, how recently you were diagnosed, whether you're on pirfenidone or nintedanib, and whether you have other lung diseases besides IPF.
What types of trials are currently open
- Treatment trials — Testing new anti-fibrotic drugs against pirfenidone or nintedanib, measuring lung function decline over 12 to 18 months.
- Combination trials — Studies adding a new drug to pirfenidone or nintedanib, or testing the two approved drugs together.
- Inhaled therapy trials — Testing drugs delivered by nebulizer or inhaler that target lung tissue directly.
- Biomarker studies — Collecting blood and lung samples to find markers that predict disease progression and treatment response.
- Quality of life trials — Testing treatments for breathlessness, cough, fatigue, and other symptoms that affect daily function.
Recently added Idiopathic Pulmonary Fibrosis trials
Real-world Study of Nerandomilast in Patients With Idiopathic Pulmonary Fibrosis and Progressive Pulmonary Fibrosis
Nerandomilast is an oral medicine that slows down the loss of functional residual capacity (FVC) in people with idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF). It was tested in two large clinical trials in which participants were carefully selected. In everyday practice, patients are often older, have more other illnesses, or take additional medicines, and it is not known whether the benefits seen in the trials also occur outside them. The Real-world Study of Nerandomilast (ReaSoN-IPF/PPF) follows people with IPF or PPF who begin nerandomilast as part of their usual medical care at several medical centers in Israel. The study does not assign any treatment; the treating physician makes every decision about therapy. The main goal is to find out whether lung function declines more slowly once nerandomilast is started. Because patients have breathing tests before they begin the medicine as well as during treatment, each patient is compared with themselves: the rate at which their FVC was declining beforehand is compared with the rate following treatment. The study also asks how patients feel, using a questionnaire called the K-BILD, and records side effects, hospital admissions, disease exacerbations, and whether patients stop the medicine. Participants attend their normal clinic visits. They complete the questionnaire before treatment starts and again at 6 to 12 months, and their routine lung function results are collected. No extra tests or visits are required beyond the questionnaire.
Try breathing exercises and pressure techniques to improve lung strength
Restrictive lung diseases (RLD) represent a significant cause of morbidity, often necessitating intensive interventions such as mechanical ventilation due to diminished lung compliance (LC) and reduced total lung capacity (TLC). Mechanical ventilation, while vital, is associated with ventilator-induced diaphragmatic dysfunction (VIDD), leading to rapid muscle atrophy and impeding patient recovery. This proposed study aims to evaluate the clinical impact of IMT and PEP on improving respiratory muscle strength, lung compliance and mobilizing secretions (effective cough reflex) in patients with RLD who require mechanical ventilation.
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