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Condition Guide

New Treatments & Clinical Trials for Pulmonary Fibrosis

Last updated June 2026Data from ClinicalTrials.gov363 active trials
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Pulmonary fibrosis causes scarring in the lungs that gradually makes breathing harder. About 200,000 people in the US have the condition. Two drugs — nintedanib and pirfenidone — can slow its progression but don't reverse damage or work for everyone.

What's actually going on in research

Trials are testing drugs that target different scarring pathways, combination therapies pairing existing drugs with new ones, and treatments aimed at specific subtypes like connective tissue disease-related fibrosis. Researchers are also studying lung transplant alternatives and ways to predict which patients will progress quickly.

Anti-fibrotic combinations

Studies are pairing nintedanib or pirfenidone with drugs that work through different mechanisms, hoping combinations will slow scarring more than either drug alone. Early trials are testing inhaled versions that deliver medication directly to lung tissue.

Autoimmune fibrosis

Trials are testing drugs for fibrosis linked to conditions like scleroderma and rheumatoid arthritis, where immune targeting may help more than in idiopathic forms. Tocilizumab recently showed benefit in scleroderma-related lung disease.

Regenerative approaches

Small trials are testing stem cells and other therapies aimed at repairing damaged lung tissue rather than just slowing scarring. Results so far have been mixed and these remain experimental.

What to know before you search

Eligibility typically depends on fibrosis type, lung function test results, oxygen needs, time since diagnosis, and whether you've taken nintedanib or pirfenidone before.

What types of trials are currently open

  • Treatment trialsTesting new anti-fibrotic drugs or combinations, usually comparing lung function decline over 12 to 24 months against existing treatments or placebo.
  • Subtype-specific trialsStudying treatments for fibrosis caused by connective tissue diseases, hypersensitivity pneumonitis, or other specific triggers where targeted therapies may work better.
  • Symptom trialsTesting treatments for shortness of breath, cough, and fatigue that often limit daily activities even when scarring progresses slowly.
  • Biomarker studiesCollecting blood, lung fluid, or imaging data to find markers that predict which patients will worsen quickly and need more aggressive treatment.

Recently added Pulmonary Fibrosis trials

RecruitingObservational study

Share your medical data to help understand antibiotic dosing in cystic fibrosis

Cystic fibrosis (CF) is associated with major pharmacokinetic and pharmacodynamic alterations affecting antibiotic exposure, including changes in absorption, distribution, metabolism, and elimination. Historically, these alterations justified the use of higher antibiotic doses in CF patients in order to achieve therapeutic concentrations and improve pulmonary outcomes. The advent of highly effective CFTR modulators, particularly the triple combination elexacaftor/tezacaftor/ivacaftor (ETI), has substantially improved pulmonary function, nutritional status, inflammatory burden, and quality of life in patients with CF. ETI therapy also appears to modify respiratory microbiology and reduce the frequency of pulmonary exacerbations. These clinical and physiological improvements may alter antibiotic pharmacokinetics and pharmacodynamics in patients with CF, potentially making current high-dose antibiotic recommendations less appropriate for some patients. Since repeated exposure to high-dose antibiotics is associated with cumulative toxicities, particularly aminoglycoside-related ototoxicity and nephrotoxicity, reassessment of antibiotic dosing strategies is warranted. The PKCF study is a multicenter, prospective, observational, non-interventional study designed to characterize the pharmacokinetic profiles of intravenous antibiotics administered during pulmonary exacerbations in adolescents and adults with cystic fibrosis receiving ETI therapy.

Roscoff, France
RecruitingObservational study

Better Tests- Improving Diagnostic Accuracy and Prognostication in Interstitial Lung Diseases

Patients with interstitial lung diseases (ILD) suffer significant delays in obtaining a diagnosis, and objective evidence of support for immunosuppressive therapies, due to weaknesses in the diagnostic tests available in ILD clinics. This project will assess several non-invasive assessments, focusing on blood and breath based sampling, to identify candidate tests that may translate through to routine clinical use. The tests being assessed will be initially employed in Pigeon Fanciers, a group recognised to be at high risk of developing ILD compared to age matched controls, due to their regular exposure to a known cause of ILD. Candidate tests will also be assessed for change over time in this group. Tests which show promise will then be utilised in patients with ILD diagnoses, including those with severe respiratory failure, to assess their acceptability to patients and their ability to provide useful prognostic information, to allow powering of future definitive clinical trials.

Blackpool, United Kingdom +2 more
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