Disease · MONDO_0002771
pulmonary fibrosis
6 sourcesdiseases-2026-09-18-0.1.0
Cite this record
ConductScience Library. pulmonary fibrosis. Diseases, record MONDO_0002771. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0002771
On this page
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.
- Highest stage of its drugs
- Approval, for 2 of its 56 drugs, from DailyMed and EMA Human Drugs
- Open Targets
Drugs56
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 | EMA Human Drugs | 7 | 4 | 4 | Drugs and targets |
| PREDNISOLONE SODIUM PHOSPHATE | CHEMBL1201014 | Small molecule | Approval | DailyMed | 1 | 0 | 0 | Drugs and targets |
| PIRFENIDONE | CHEMBL1256391 | Small molecule | Phase 3 | ClinicalTrials.gov | 8 | 8 | 8 | Drugs and targets |
| TREPROSTINIL | CHEMBL1237119 | Small molecule | Phase 3 | ClinicalTrials.gov | 3 | 3 | 3 | Drugs and targets |
| CYCLOPHOSPHAMIDE | CHEMBL1200796 | Small molecule | Phase 3 | ClinicalTrials.gov | 2 | 2 | 2 | Drugs and targets |
| ADMILPARANT | CHEMBL5087506 | Small molecule | Phase 3 | ClinicalTrials.gov | 3 | 1 | 1 | Drugs and targets |
| TADALAFIL | CHEMBL779 | Small molecule | Phase 3 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets |
| ILOPROST | CHEMBL494 | Small molecule | Phase 2/3 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets |
| PYRUVATE SODIUM | CHEMBL181886 | Small molecule | Phase 2/3 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets |
| DANAZOL | CHEMBL1479 | Small molecule | Phase 2 | ClinicalTrials.gov | 3 | 3 | 3 | Drugs and targets |
1 to 10 of 56
See all 56 drugsTargets84
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 | 6 | Glucocorticoid receptor agonist | Approval | https://conductscience.org/library/genes/2908 | Drugs and targets |
| PDGFRB | Platelet-derived growth factor receptor | 3 | Platelet-derived growth factor receptor beta inhibitor; Platelet-derived growth factor receptor inhibitor | Approval | https://conductscience.org/library/genes/5159 | Drugs and targets |
| AGTR1 | Type-1 angiotensin II receptor | 2 | Type-1 angiotensin II receptor antagonist | Phase 1/2 | https://conductscience.org/library/genes/185 | Drugs and targets |
| FGFR1 | Fibroblast growth factor receptor | 2 | Fibroblast growth factor receptor inhibitor | Approval | https://conductscience.org/library/genes/2260 | Drugs and targets |
| FGFR2 | Fibroblast growth factor receptor | 2 | Fibroblast growth factor receptor inhibitor | Approval | https://conductscience.org/library/genes/2263 | Drugs and targets |
| FGFR3 | Fibroblast growth factor receptor | 2 | Fibroblast growth factor receptor inhibitor | Approval | https://conductscience.org/library/genes/2261 | Drugs and targets |
| FGFR4 | Fibroblast growth factor receptor | 2 | Fibroblast growth factor receptor inhibitor | Approval | https://conductscience.org/library/genes/2264 | Drugs and targets |
| FLT1 | Vascular endothelial growth factor receptor | 2 | Vascular endothelial growth factor receptor inhibitor | Approval | https://conductscience.org/library/genes/2321 | Drugs and targets |
| FLT4 | Vascular endothelial growth factor receptor | 2 | Vascular endothelial growth factor receptor inhibitor | Approval | https://conductscience.org/library/genes/2324 | Drugs and targets |
| IL13 | Interleukin-13 | 2 | Interleukin-13 inhibitor | Phase 2 | https://conductscience.org/library/genes/3596 | Drugs and targets |
1 to 10 of 84
See all 84 targetsTrials47
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. 2 more are not shown, since the checks do not confirm them.
| Trial | Title | Phase | Participants | Results first posted | Conditions | Drugs | Linked by | Source |
|---|---|---|---|---|---|---|---|---|
| NCT02633293 | An Open Label Extension Study to Evaluate Inhaled Treprostinil in Adult PH With ILD Including CPFE | Phase 2/3 | 243 | 2022-12-02 | Pulmonary Hypertension, Interstitial Lung Disease, Combined Pulmonary Fibrosis and Emphysema | TREPROSTINIL | Open Targets' reading of its record | ClinicalTrials.gov |
| NCT00004563 | Scleroderma Lung Disease | Phase 3 | 158 | 2015-03-06 | Lung Diseases, Pulmonary Fibrosis, Systemic Scleroderma, Scleroderma, Systemic | CYCLOPHOSPHAMIDE | Its record names the disease | ClinicalTrials.gov |
| NCT00883129 | Comparison of Therapeutic Regimens for Scleroderma Interstitial Lung Disease (The Scleroderma Lung Study II) | Phase 2 | 142 | 2017-02-10 | Scleroderma, Interstitial Lung Disease | CYCLOPHOSPHAMIDE, MYCOPHENOLATE MOFETIL | Its record names the disease | ClinicalTrials.gov |
| NCT04334460 | Safety and Antiviral Activity of BLD-2660 in COVID-19 Hospitalized Subjects | Phase 2 | 120 | 2022-04-06 | Sars-CoV-2, Covid19 | DAZCAPISTAT | Its record names the disease | ClinicalTrials.gov |
| NCT00080223 | Safety Study of Oral Pirfenidone in Patients With Pulmonary Fibrosis/Idiopathic Pulmonary Fibrosis | Phase 2 | 83 | 2016-03-09 | Idiopathic Pulmonary Fibrosis, Pulmonary Fibrosis | PIRFENIDONE | Its record names the disease | ClinicalTrials.gov |
| NCT05285982 | A Study to Evaluate Long-term Safety of Nintedanib in Children and Adolescents With Interstitial Lung Disease (InPedILD®-ON) | Phase 3 | 54 | 2026-03-04 | Lung Diseases, Interstitial | NINTEDANIB | Open Targets' reading of its record | ClinicalTrials.gov |
| NCT00001596 | Oral Pirfenidone for the Pulmonary Fibrosis of Hermansky-Pudlak Syndrome | Phase 2 | 35 | 2012-01-26 | Albinism, Inborn Errors of Metabolism, Oculocutaneous Albinism, Platelet Storage Pool Deficiency, Pulmonary Fibrosis | PIRFENIDONE | Its record names the disease | ClinicalTrials.gov |
| NCT04824365 | Sodium Pyruvate Nasal Spray Treatment of COVID-19 Infection | Phase 2/3 | 30 | 2022-09-26 | COVID-19 | PYRUVATE SODIUM | Its record names the disease | ClinicalTrials.gov |
| NCT01441037 | Danazol for Genetic Bone Marrow and Lung Disorders | Phase 1/2 | 27 | 2018-07-11 | Aplastic Anemia | DANAZOL | Open Targets' reading, confirmed by NLM's indexing of its papers | ClinicalTrials.gov |
| NCT02567799 | BIO 300 Non-Small Cell Lung Cancer Study | Phase 1/2 | 21 | 2023-12-20 | Carcinoma, Non-Small-Cell Lung | CARBOPLATIN, GENISTEIN, PACLITAXEL | Its record names the disease | ClinicalTrials.gov |
1 to 10 of 47
See all 47 trialsComparisons in its trials9
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 |
|---|---|---|---|---|---|
| Cylophosphamide vs Placebo: serious adverse events | 5 of 79 | 5 of 79 | 158 | NCT00004563 | Trial comparisons |
| Mycophenolate Arm vs Cyclophosphamide Arm: serious adverse events | 27 of 69 | 22 of 73 | 142 | NCT00883129 | Trial comparisons |
| Active Group vs Placebo Group: deaths from any cause | 2 of 77 | 2 of 38 | 115 | NCT04334460 | Trial comparisons |
| Active Group vs Placebo Group: serious adverse events | 9 of 77 | 9 of 38 | 115 | NCT04334460 | Trial comparisons |
| Pirfenidone vs Placebo: serious adverse events | 6 of 23 | 2 of 12 | 35 | NCT00001596 | Trial comparisons |
| Treatment of COVID-19 Infected Patients With a Sodium Pyruvate Nasal Spray vs Placebo Control Treatment of COVID-19 Infected Patients: deaths from any cause | 0 of 15 | 0 of 15 | 30 | NCT04824365 | Trial comparisons |
| Treatment of COVID-19 Infected Patients With a Sodium Pyruvate Nasal Spray vs Placebo Control Treatment of COVID-19 Infected Patients: serious adverse events | 0 of 15 | 0 of 15 | 30 | NCT04824365 | Trial comparisons |
| Bortezomib Plus Mycophenolate Mofetil vs Placebo Plus Mycophenolate Mofetil: deaths from any cause | 0 of 3 | 0 of 4 | 7 | NCT02370693 | Trial comparisons |
| Bortezomib Plus Mycophenolate Mofetil vs Placebo Plus Mycophenolate Mofetil: serious adverse events | 1 of 3 | 1 of 4 | 7 | NCT02370693 | Trial comparisons |
Papers from its trials75
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/MONDO_0002771
GET https://conductscience.org/api/diseases/v1/MONDO_0002771/drugs?page=2