Disease · EFO_0000768
idiopathic pulmonary fibrosis
6 sourcesdiseases-2026-09-18-0.1.0
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ConductScience Library. idiopathic pulmonary fibrosis. Diseases, record EFO_0000768. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/EFO_0000768
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Disease
Names and IDs as Open Targets gives them. Its IDs in MeSH, Orphanet, the Disease Ontology and the NCI Thesaurus are those Open Targets states for it.
- Other names
- CFA, UIP, cryptogenic fibrosing alveolitis, fibrocystic pulmonary dysplasia, idiopathic pulmonary fibrosis, familial, usual interstitial pneumonia
- Open Targets
- Highest stage of its drugs
- Approval, for 5 of its 133 drugs, from ClinicalTrials.gov, DailyMed, EMA Human Drugs, FDA, PMDA and TTD
- Open Targets
Drugs133
The drugs Open Targets' clinical reports study for the disease, the highest stage first, then the most trial reports. The stage is Open Targets', the highest any of its sources reports for the pair, and each row names the sources that report it.
| Drug | ChEMBL ID | Type | Open Targets' stage | Stage reported by | Reports | Of them trials | Trials shown here | Source |
|---|---|---|---|---|---|---|---|---|
| NINTEDANIB | CHEMBL502835 | Small molecule | Approval | ClinicalTrials.gov, EMA Human Drugs and TTD | 36 | 31 | 31 | Drugs and targets |
| PIRFENIDONE | CHEMBL1256391 | Small molecule | Approval | ClinicalTrials.gov, DailyMed, EMA Human Drugs, PMDA and TTD | 33 | 27 | 27 | Drugs and targets |
| INTERFERON GAMMA-1B | CHEMBL1201564 | Protein | Approval | TTD | 8 | 7 | 7 | Drugs and targets |
| PREDNISOLONE SODIUM PHOSPHATE | CHEMBL1201014 | Small molecule | Approval | DailyMed and FDA | 8 | 0 | 0 | Drugs and targets |
| NINTEDANIB ESYLATE | CHEMBL3039504 | Small molecule | Approval | DailyMed and PMDA | 2 | 0 | 0 | Drugs and targets |
| NERANDOMILAST | CHEMBL5314532 | Small molecule | Preapproval | EMA Human Drugs | 7 | 5 | 5 | Drugs and targets |
| PREDNISONE | CHEMBL635 | Small molecule | Phase 3 | ClinicalTrials.gov | 7 | 7 | 6 | Drugs and targets |
| METHYLPREDNISOLONE | CHEMBL650 | Small molecule | Phase 3 | ClinicalTrials.gov | 6 | 6 | 5 | Drugs and targets |
| SILDENAFIL | CHEMBL192 | Small molecule | Phase 3 | ClinicalTrials.gov | 6 | 6 | 6 | Drugs and targets |
| PAMREVLUMAB | CHEMBL2109377 | Antibody | Phase 3 | ClinicalTrials.gov | 5 | 5 | 5 | Drugs and targets |
1 to 10 of 133
See all 133 drugsTargets157
The targets ChEMBL's mechanism records give for the disease's drugs, through Open Targets, the most drugs first. A mechanism record says what a drug acts on, not that the target causes the disease.
| Target | Protein | Its drugs that act on it | Mechanisms | Highest stage of those drugs | Gene in Genes | Source |
|---|---|---|---|---|---|---|
| NR3C1 | Glucocorticoid receptor | 10 | Glucocorticoid receptor agonist | Approval | https://conductscience.org/library/genes/2908 | Drugs and targets |
| IL13 | Interleukin-13 | 4 | Interleukin-13 inhibitor | Phase 2 | https://conductscience.org/library/genes/3596 | Drugs and targets |
| PDGFRB | Platelet-derived growth factor receptor | 4 | Platelet-derived growth factor receptor beta inhibitor; Platelet-derived growth factor receptor inhibitor | Approval | https://conductscience.org/library/genes/5159 | Drugs and targets |
| ABL1 | Bcr/Abl fusion protein | 3 | Bcr/Abl fusion protein inhibitor; Tyrosine-protein kinase ABL inhibitor | Phase 2/3 | https://conductscience.org/library/genes/25 | Drugs and targets |
| CSF3R | Granulocyte colony-stimulating factor receptor | 3 | Granulocyte colony stimulating factor receptor agonist | Phase 1/2 | https://conductscience.org/library/genes/1441 | Drugs and targets |
| EDNRA | Endothelin receptor, ET-A/ET-B | 3 | Endothelin receptor, ET-A/ET-B antagonist | Phase 3 | https://conductscience.org/library/genes/1909 | Drugs and targets |
| EDNRB | Endothelin receptor, ET-A/ET-B | 3 | Endothelin receptor, ET-A/ET-B antagonist | Phase 3 | https://conductscience.org/library/genes/1910 | Drugs and targets |
| FLT4 | Vascular endothelial growth factor receptor | 3 | Vascular endothelial growth factor receptor 3 inhibitor; Vascular endothelial growth factor receptor inhibitor | Approval | https://conductscience.org/library/genes/2324 | Drugs and targets |
| KDR | Vascular endothelial growth factor receptor | 3 | Vascular endothelial growth factor receptor 2 inhibitor; Vascular endothelial growth factor receptor inhibitor | Approval | https://conductscience.org/library/genes/3791 | Drugs and targets |
| PTGIR | Prostacyclin receptor | 3 | Prostanoid IP receptor agonist | Phase 3 | https://conductscience.org/library/genes/5739 | Drugs and targets |
1 to 10 of 157
See all 157 targetsTrials198
Trials an Open Targets clinical report links to the disease, posted results first, then the largest. A trial is shown when the release's checks confirm its link to the disease and Drugs and targets shows its link to one of the disease's drugs. 8 more are not shown, since the checks do not confirm them.
| Trial | Title | Phase | Participants | Results first posted | Conditions | Drugs | Linked by | Source |
|---|---|---|---|---|---|---|---|---|
| NCT03958071 | A Study Based on Medical Records That Looks at the Characteristics of Idiopathic Pulmonary Fibrosis Patients Grouped by the Type of Medication They Are Taking | 13,264 | 2020-12-17 | Idiopathic Pulmonary Fibrosis | NINTEDANIB, PIRFENIDONE | Its record names the disease | ClinicalTrials.gov | |
| NCT02607722 | All-Case Surveillance of Ofev in Patients With IPF in Japan | 10,117 | 2024-10-15 | Idiopathic Pulmonary Fibrosis | NINTEDANIB | Its record names the disease | ClinicalTrials.gov | |
| NCT05779007 | Dose Reduction and Discontinuation With Anti-Fibrotic Medications | 2,778 | 2025-07-28 | Idiopathic Pulmonary Fibrosis | NINTEDANIB, PIRFENIDONE | Its record names the disease | ClinicalTrials.gov | |
| NCT05870956 | Burden of Nintedanib Non-adherence Among Idiopathic Pulmonary Fibrosis (IPF) Patients | 1,798 | 2024-10-01 | Idiopathic Pulmonary Fibrosis | NINTEDANIB | Its record names the disease | ClinicalTrials.gov | |
| NCT05321069 | A Study to Find Out Whether BI 1015550 Improves Lung Function in People With Idiopathic Pulmonary Fibrosis (IPF) | Phase 3 | 1,177 | 2025-09-12 | Idiopathic Pulmonary Fibrosis | NERANDOMILAST | Its record names the disease | ClinicalTrials.gov |
| NCT00662038 | Open-Label Study of the Long Term Safety of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis (IPF) | Phase 3 | 1,058 | 2017-03-29 | Idiopathic Pulmonary Fibrosis | PIRFENIDONE | Its record names the disease | ClinicalTrials.gov |
| NCT03733444 | A Clinical Study to Test How Effective and Safe GLPG1690 is for Participants With Idiopathic Pulmonary Fibrosis (IPF) When Used Together With Standard of Care | Phase 3 | 781 | 2022-07-29 | Idiopathic Pulmonary Fibrosis | ZIRITAXESTAT | Its record names the disease | ClinicalTrials.gov |
| NCT01619085 | Extension Trial of the Long Term Safety of BIBF 1120 in Patients With Idiopathic Pulmonary Fibrosis | Phase 3 | 752 | 2018-07-26 | Idiopathic Pulmonary Fibrosis | NINTEDANIB | Its record names the disease | ClinicalTrials.gov |
| NCT04552899 | A Study to Evaluate the Efficacy and Safety of Recombinant Human Pentraxin-2 (rhPTX-2; PRM-151) in Participants With Idiopathic Pulmonary Fibrosis | Phase 3 | 665 | 2024-04-18 | Idiopathic Pulmonary Fibrosis | ZINPENTRAXIN ALFA | Its record names the disease | ClinicalTrials.gov |
| NCT00391443 | BUILD 3: Bosentan Use in Interstitial Lung Disease | Phase 3 | 616 | 2012-05-25 | Idiopathic Pulmonary Fibrosis | BOSENTAN | Its record names the disease | ClinicalTrials.gov |
1 to 10 of 198
See all 198 trialsComparisons in its trials91
Comparisons Trial comparisons calculated in the trials shown here, most participants first. A comparison in one of these trials does not always compare a treatment for the disease, so read the two groups.
| Comparison | Group 1, with the outcome | Group 2, with the outcome | Participants | Trial | Source |
|---|---|---|---|---|---|
| Nintedanib Initiators Cohort vs Pirfenidone Initiators Cohort: Number of Patients With Dose Reduction and/or Temporary Dose Reduction (Sub-optimal Dose) by 12 Months | 301 of 1,389 | 373 of 1,389 | 2,778 | NCT05779007 | Trial comparisons |
| Zinpentraxin Alfa vs Placebo: deaths from any cause | 4 of 331 | 4 of 333 | 664 | NCT04552899 | Trial comparisons |
| Zinpentraxin Alfa vs Placebo: Percentage of Participants With Adverse Events (AEs) | 247 of 331 | 238 of 329 | 660 | NCT04552899 | Trial comparisons |
| Zinpentraxin Alfa vs Placebo: Percentage of Participants With Infusion-related Reactions (IRRs) and Other Adverse Events of Special Interest | 12 of 331 | 5 of 329 | 660 | NCT04552899 | Trial comparisons |
| Zinpentraxin Alfa vs Placebo: Percentage of Participants Permanently Discontinuing Study Treatment Due to AEs | 9 of 331 | 6 of 329 | 660 | NCT04552899 | Trial comparisons |
| Zinpentraxin Alfa vs Placebo: serious adverse events | 46 of 331 | 40 of 329 | 660 | NCT04552899 | Trial comparisons |
| Bosentan vs Placebo: serious adverse events | 129 of 406 | 74 of 209 | 615 | NCT00391443 | Trial comparisons |
| Active Arm vs Placebo Arm: serious adverse events | 55 of 278 | 69 of 277 | 555 | NCT01366209 | Trial comparisons |
| Nintedanib 150mg Bid vs Placebo: serious adverse events | 98 of 329 | 72 of 219 | 548 | NCT01335477 | Trial comparisons |
| Simtuzumab vs Simtuzumab Placebo: deaths from any cause | 31 of 271 | 32 of 272 | 543 | NCT01769196 | Trial comparisons |
1 to 10 of 91
See all 91 comparisons in its trialsPapers from its trials176
The papers the trials shown here list on ClinicalTrials.gov as their results (RESULT) or as drawn from them (DERIVED), results first, newest first.
Data dates
The date of each source this release read.
Sources 6
- ClinicalTrials.gov's posted results, through CTTI's AACTFreely available. Acknowledgement requested
- ChEMBL, through Open TargetsCC BY-SA 3.0
- ClinicalTrials.govNo license stated
- NLM's MeSHNLM's terms
- Open TargetsCC0 1.0
- PubMedNLM's terms
Each source's terms
- ClinicalTrials.gov's posted results, through CTTI's AACTClinicalTrials.gov data via Aggregate Analysis of ClinicalTrials.gov (AACT), CTTI. CTTI makes its materials freely available and requests acknowledgement. https://aact.ctti-clinicaltrials.org/documentation/299
- ChEMBL, through Open TargetsCC BY-SA 3.0, for the parts from ChEMBL. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
- ClinicalTrials.govnone stated
- NLM's MeSHNLM's
- Open TargetsCC0 1.0. Open Targets asks that users cite its latest paper: Buniello et al. 2025, Open Targets Platform: facilitating therapeutic hypotheses building in drug discovery, Nucleic Acids Research 53(D1):D1467, PMID 39657122. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
- PubMedNLM's terms
ClinicalTrials.gov's data carry an international copyright outside the United States: https://clinicaltrials.gov/about-site/terms-conditions.
For software
Read this disease from software.
The record and each of its lists are JSON, twenty rows a page. No key.
GET https://conductscience.org/api/diseases/v1/EFO_0000768
GET https://conductscience.org/api/diseases/v1/EFO_0000768/drugs?page=2