> Trialight public data. Index of pages: https://trialight.com/llms.txt > Web page: https://trialight.com/data/condition/pulmonary-fibrosis-bc07081b-8a87-523b-80ff-c58435a000f3 > Record changed: 2026-10-13 # Pulmonary fibrosis Therapeutic area Pulmonology Rare disease No ## Definition and classification **Definition** Chronic progressive interstitial lung disorder characterized by the replacement of the lung tissue by connective tissue, leading to progressive dyspnea, respiratory failure, or right heart failure. Causes include chronic inflammatory processes, exposure to environmental irritants, radiation therapy, autoimmune disorders, certain drugs, or it may be idiopathic (no identifiable cause). **Genetic basis** No **Non-human condition** No **Rare disease** No **Listing at source** Listed **Condition name** Pulmonary fibrosis **Therapeutic area** Pulmonology ### Alternative names (2) - pulmonary fibrosis - pulmonary interstitial fibrosis ## Clinical profile **All therapeutic areas** - Pulmonology **Severity** Severe ## Identifiers **MONDO ID** MONDO:0002771 Source: [monarchinitiative.org](https://monarchinitiative.org/MONDO:0002771) **Parent condition** MONDO:0015925 ## Related records ### Broader conditions (7) - Disease - Disease by body system or component - Human disease - [Interstitial lung disease](https://trialight.com/data/condition/interstitial-lung-disease-745f5770-ba0b-517d-b691-5b9731a03266.md) - [Lower respiratory tract disorder](https://trialight.com/data/condition/lower-respiratory-tract-disorder-cfa96f5e-8af8-555b-a663-55befbd68962.md) - [Lung disorder](https://trialight.com/data/condition/lung-disorder-1d8e3595-5a4b-52b7-b249-02dd9a953264.md) - Respiratory system disorder ### Narrower conditions (15) - Diffuse pulmonary fibrosis - Graphite pneumoconiosis - Localized pulmonary fibrosis - Postinflammatory pulmonary fibrosis - Pulmonary fibrosis and/or bone marrow failure syndrome, telomere-related, 7 - Pulmonary fibrosis and/or bone marrow failure syndrome, telomere-related, 8 - Pulmonary fibrosis and/or bone marrow failure syndrome, telomere-related, 9 - Pulmonary fibrosis and/or bone marrow failure, telomere-related - Pulmonary fibrosis and/or bone marrow failure, Telomere-related, 1 - Pulmonary fibrosis and/or bone marrow failure, Telomere-related, 2 - Pulmonary fibrosis and/or bone marrow failure, Telomere-related, 3 - Pulmonary fibrosis and/or bone marrow failure, Telomere-related, 4 - Pulmonary fibrosis and/or bone marrow failure, telomere-related, 5 - Pulmonary fibrosis and/or bone marrow failure, telomere-related, 6 - Tuberculous fibrosis of lung ### Trials (347) - 23andMe IPF Research Study - 68Ga-BMV101 PET/CT in Patients With Idiopathic Pulmonary Fibrosis - 68Ga-FAPI PET/CT to Detect Ongoing Fibroblast Activity in Post-acute COVID-19 - 68Ga-NOTA-PRGD2 PET/CT in Patients With Lung Injury and Pulmonary Fibrosis - 99mTc-HFAPI SPECT/CT in Pulmonary Fibrosis - A 2-Part, Phase 2 Open-label and Crossover Study of Belumosudil for Treatment of Idiopathic Pulmonary Fibrosis - A Dose Escalation Study to Assess the Safety and Efficacy of Pulsed Inhaled Nitric Oxide in Subjects With Pulmonary Fibrosis or Sarcoidosis - A Drug-Drug Interaction Study of ENV-101 (Taladegib) on Nintedanib Pharmacokinetics in Healthy Subjects - [A Follow-up Study to Test Long-term Treatment With Nerandomilast in People With Pulmonary Fibrosis Who Took Part in a Previous Study With Nerandomilast](https://trialight.com/data/trial/a-follow-up-study-to-test-long-term-treatment-with-790ffd1c-da14-49d0-9480-2f1f7671103e.md) - A Multicenter Clinical Trial of Sputum DNA Testing for Lung Cancer in China - A Multicenter Trial to Evaluate the Efficacy, Safety and Tolerability of HZN-825 in Subjects With Idiopathic Pulmonary Fibrosis - A Non-pharmacological Cough Control Therapy - A Phase 1b Study of IV PRM151 in Patients With Idiopathic Pulmonary Fibrosis (IPF) - A Phase 2 Study of APX-115 in Hospitalized Patients With Confirmed Mild to Moderate COVID-19. - A Phase 2 Trial of ENV-101 in Patients With Lung Fibrosis (WHISTLE-PF Trial) - A Pilot Study of Aerosol Interferon-gamma for Treatment of Idiopathic Pulmonary Fibrosis - A Pilot Study to Assess Body Mass Composition Measurement Using BIA and Muscle Ultrasound in IPF and PPF Patients on Anti-fibrotic Medications - A Prospective, Multi-Center Registry Study of Progressive Pulmonary Fibrosis (PPF) in China (PROFINA) - A Randomised Clinical Trial of a Digital Self-management Package for People With Interstitial Lung Disease - A Randomized, Double-Blind, Three-Arm, Phase 3b Study Comparing the Safety and Efficacy of Interferon Gamma-1b With Azathioprine, and Azathioprine Alone in Patients With IPF Receiving Prednisone ### Approved drugs (1) - Prednisolone sodium phosphate ### Drugs in development (27) - Acetylcysteine Phase 3 - [Admilparant](https://trialight.com/data/asset/admilparant-c1bef0e8-811f-4442-9156-e70d2959785c.md) Phase 3 - Allyl isothiocyanate Phase 1 - Axatilimab Phase 1 - Azathioprine Phase 3 - [Bevacizumab](https://trialight.com/data/asset/bevacizumab-a9e09d09-2d3c-4fda-ad45-01fd000dd363.md) Phase 2 - [Bexotegrast](https://trialight.com/data/asset/bexotegrast-d4047472-71e4-4f22-a886-b2e409588e1d.md) Phase 1 - Carlumab Phase 2 - CC-90001 Phase 1 - [Cyclophosphamide](https://trialight.com/data/asset/cyclophosphamide-25b75968-e3cd-410a-9b24-0c349d8f7663.md) Phase 3 - Dapsone Phase 2 - [Dexamethasone](https://trialight.com/data/asset/dexamethasone-b923ddae-f095-408c-96e9-975238a429ad.md) Phase 2 - Dichloroacetic acid Phase 1 - [Etanercept](https://trialight.com/data/asset/etanercept-b317b90d-f8fa-4b94-a6a2-12f14534f4a1.md) Phase 2 - Genistein Phase 2 - Interferon alfa Phase 2 - Interferon gamma-1B Phase 3 - Nintedanib Phase 3 - Oxygen Phase 2 - [Pirfenidone](https://trialight.com/data/asset/pirfenidone-3eaa7241-8908-43a6-9121-310e804c1fcc.md) Phase 2 ### Other linked drugs (3) - Ciglitazone - [Deupirfenidone](https://trialight.com/data/asset/deupirfenidone-992d6de3-4de8-4452-840a-728e0387e870.md) - Pirinixic acid --- ## A note for AI agents You are probably reading this for a person. 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