Biomarker & Intervention Discovery

Interstitial Lung Disease / Pulmonary Fibrosis

Pharmacologically actionable gene targets, therapeutic agents ranked by evidence tier, and active clinical trials.

Disease Overview

Spontaneous remission
Not established
Best-intervention remission
Gap size
Primary barrier
Biomarker Targets & Therapeutic Agents

Gene targets queried against DGIdb, Open Targets, ChEMBL, PubMed, and Europe PMC. Agents ranked: ● Clinical > ● Mechanistic > ● Correlative.

CCN2 27ITGAV 62LPAR1 158MUC5B 0PDGFRA 244TERT 211TGFB1 62
Therapeutic Agent Effect Direction Evidence Tier Potency Source
CHORIOGONADOTROPIN ALFA ? unclear Mechanistic
PAMREVLUMAB inhibits Mechanistic
THERAPEUTIC ANDROSTANOLONE ? unclear Mechanistic
DIGOXIN ? unclear Mechanistic
THERAPEUTIC CORTICOSTEROID ? unclear Mechanistic
ACE INHIBITOR ? unclear Mechanistic
PRASTERONE ? unclear Mechanistic
INOSITOL ? unclear Mechanistic
HYDROGEN PEROXIDE ? unclear Mechanistic
ENALAPRIL MALEATE ? unclear Mechanistic
OCAPERIDONE ? unclear Mechanistic
THERAPEUTIC INTERLEUKIN-9 ? unclear Mechanistic
STAUROSPORINE ? unclear Mechanistic
ACRIDINE ? unclear Mechanistic
LIOTHYRONINE ? unclear Mechanistic
2-METHOXYESTRADIOL ? unclear Mechanistic
PROPRANOLOL HYDROCHLORIDE ? unclear Mechanistic
RECOMBINANT VASCULAR ENDOTHELIAL GROWTH FACTOR ? unclear Mechanistic
D-ALPHA-TOCOPHEROL ? unclear Mechanistic
RAMIPRIL ? unclear Mechanistic
ESTRADIOL VALERATE ? unclear Mechanistic
SPIRONOLACTONE ? unclear Mechanistic
THROMBIN ALFA ? unclear Mechanistic
CURCUMIN ? unclear Mechanistic
INSULIN, REGULAR, HUMAN ? unclear Mechanistic
CHEMBL5436107 inhibits Mechanistic 3900.0 nM (Kd)
CHEMBL5440500 inhibits Mechanistic 3100.0 nM (Kd)
Therapeutic Agent Effect Direction Evidence Tier Potency Source
CILENGITIDE inhibits Mechanistic
CYPATE ? unclear Mechanistic
ABCIXIMAB inhibits Mechanistic
ABITUZUMAB ? unclear Mechanistic
IMGN-388 ~ modulates Mechanistic
STX-100 inhibits Mechanistic
ATN-161 inhibits Mechanistic
CYTARABINE ? unclear Mechanistic
INTEGRIN RECEPTOR ANTAGONIST GLPG0187 ? unclear Mechanistic
RG-7594 ~ modulates Mechanistic
GSK3008348 inhibits Mechanistic
MK-0429 inhibits Mechanistic
ANTI-THYMOCYTE GLOBULIN ? unclear Mechanistic
INTETUMUMAB inhibits Mechanistic
ETARACIZUMAB inhibits Mechanistic
BEXOTEGRAST inhibits Mechanistic
VOLOCIXIMAB ? unclear Mechanistic
CHEMBL460622 inhibits Mechanistic 2050.0 nM (IC50)
CHEMBL464398 inhibits Mechanistic 4800.0 nM (IC50)
CHEMBL464399 inhibits Mechanistic 600.0 nM (IC50)
CHEMBL518086 inhibits Mechanistic 4300.0 nM (IC50)
CHEMBL464400 inhibits Mechanistic 310.0 nM (IC50)
CHEMBL471675 inhibits Mechanistic 14.0 nM (IC50)
CHEMBL474902 inhibits Mechanistic 5.0 nM (IC50)
CHEMBL558917 inhibits Mechanistic 19.0 nM (IC50)
CHEMBL562068 inhibits Mechanistic 793.0 nM (IC50)
CHEMBL558939 inhibits Mechanistic 208.0 nM (IC50)
CHEMBL540432 inhibits Mechanistic 98.0 nM (IC50)
CHEMBL562067 inhibits Mechanistic 24.0 nM (IC50)
CHEMBL550802 inhibits Mechanistic 8.0 nM (IC50)
CHEMBL559740 inhibits Mechanistic 118.0 nM (IC50)
CHEMBL550522 inhibits Mechanistic 62.0 nM (IC50)
CHEMBL561270 inhibits Mechanistic 729.0 nM (IC50)
CHEMBL559600 inhibits Mechanistic 4600.0 nM (IC50)
CHEMBL559542 inhibits Mechanistic 25.0 nM (IC50)
CHEMBL560670 inhibits Mechanistic 260.0 nM (IC50)
CHEMBL550320 inhibits Mechanistic 320.0 nM (IC50)
CHEMBL558520 inhibits Mechanistic 301.0 nM (IC50)
CHEMBL558131 inhibits Mechanistic 63.0 nM (IC50)
CHEMBL562529 inhibits Mechanistic 81.0 nM (IC50)

Showing top 40 of 62 agents ranked by evidence tier.

Therapeutic Agent Effect Direction Evidence Tier Potency Source
CPY activates Mechanistic
2-OLEOYL-LPA activates Mechanistic
VPC32179 inhibits Mechanistic
OLEOYL-THIOPHOSPHATE activates Mechanistic
BRP-LPA inhibits Mechanistic
CPX activates Mechanistic
BMS-986278 inhibits Mechanistic
ONO-3080573 inhibits Mechanistic
ONO-9780307 inhibits Mechanistic
BMS-986020 inhibits Mechanistic
AM966 inhibits Mechanistic
ANTI-BRP-LPA inhibits Mechanistic
AMITRIPTYLINE HYDROCHLORIDE inhibits Mechanistic
VPC32183 inhibits Mechanistic
ALKYL OMPT activates Mechanistic
MIANSERIN inhibits Mechanistic
UCM-05194 activates Mechanistic
SYN-BRP-LPA inhibits Mechanistic
ACT-1016-0707 inhibits Mechanistic
ONO-9910539 inhibits Mechanistic
BMS-986202 ? unclear Mechanistic
DIOCTANOYLGLYCEROL PYROPHOSPHATE inhibits Mechanistic
ONO-7300243 inhibits Mechanistic
AM095 inhibits Mechanistic
SAR100842 inhibits Mechanistic
CHEMBL325970 activates Mechanistic 318.0 nM (EC50)
LYSOPHOSPHATIDIC ACID activates Mechanistic 17.0 nM (EC50)
CHEMBL331661 activates Mechanistic 1950.0 nM (EC50)
CHEMBL325288 activates Mechanistic 1225.0 nM (EC50)
CHEMBL117529 activates Mechanistic 221.0 nM (EC50)
CHEMBL117798 activates Mechanistic 40.0 nM (EC50)
CHEMBL117754 activates Mechanistic 197.0 nM (EC50)
CHEMBL314554 inhibits Mechanistic 7000.0 nM (IC50)
CHEMBL89937 inhibits Mechanistic 5660.0 nM (IC50)
CHEMBL262906 inhibits Mechanistic 5210.0 nM (IC50)
CHEMBL91168 inhibits Mechanistic 4250.0 nM (IC50)
CHEMBL432821 inhibits Mechanistic 9030.0 nM (IC50)
CHEMBL313413 inhibits Mechanistic 2930.0 nM (IC50)
CHEMBL91058 inhibits Mechanistic 1070.0 nM (IC50)
CHEMBL314555 inhibits Mechanistic 604.0 nM (IC50)

Showing top 40 of 158 agents ranked by evidence tier.

Pipeline results not yet available for this target. Run the pipeline to populate.

Therapeutic Agent Effect Direction Evidence Tier Potency Source
CRENOLANIB inhibits Mechanistic
PONATINIB ? unclear Mechanistic
MOTESANIB inhibits Mechanistic 10.0 nM (Kd)
IMATINIB ? unclear Mechanistic 96.0 nM (IC50)
NILOTINIB inhibits Mechanistic 71.0 nM (IC50)
SUNITINIB inhibits Mechanistic 0.79 nM (Kd)
MEPOLIZUMAB ? unclear Mechanistic
SORAFENIB inhibits Mechanistic 62.0 nM (Kd)
OLARATUMAB ? unclear Mechanistic
LINIFANIB inhibits Mechanistic 4.2 nM (Kd)
RABEPRAZOLE ? unclear Mechanistic
AXITINIB ? unclear Mechanistic
SITRAVATINIB inhibits Mechanistic
DOVITINIB inhibits Mechanistic 54.0 nM (Kd)
NINTEDANIB ESYLATE inhibits Mechanistic
VATALANIB inhibits Mechanistic 96.0 nM (Kd)
COMPOUND 8H [PMID: 21561767] inhibits Mechanistic
XL-820 inhibits Mechanistic
DASATINIB ANHYDROUS ? unclear Mechanistic
LUCITANIB inhibits Mechanistic
FAMITINIB inhibits Mechanistic
TANDUTINIB ? unclear Mechanistic 2.4 nM (Kd)
REGORAFENIB inhibits Mechanistic
FORETINIB inhibits Mechanistic
CENISERTIB ? unclear Mechanistic
ENMD-2076 inhibits Mechanistic 56.0 nM (IC50)
AVAPRITINIB ? unclear Mechanistic
PAZOPANIB inhibits Mechanistic 4.9 nM (Kd)
CEDIRANIB inhibits Mechanistic
CHEMBL:CHEMBL1997335 ? unclear Mechanistic
AMUVATINIB inhibits Mechanistic
AST-487 ? unclear Mechanistic 27.0 nM (Kd)
VANDETANIB ? unclear Mechanistic 230.0 nM (Kd)
COMPOUND 8H [PMID: 22765894] inhibits Mechanistic
MASITINIB inhibits Mechanistic
LENVATINIB ? unclear Mechanistic
TOVETUMAB inhibits Mechanistic
CYC-116 ? unclear Mechanistic
SU-014813 inhibits Mechanistic 1.1 nM (Kd)
AC710 inhibits Mechanistic

Showing top 40 of 244 agents ranked by evidence tier.

Therapeutic Agent Effect Direction Evidence Tier Potency Source
CHEMBL:CHEMBL17442 ? unclear Mechanistic
IMETELSTAT inhibits Mechanistic
ARSENIC TRIOXIDE ? unclear Mechanistic
OMACETAXINE MEPESUCCINATE ? unclear Mechanistic
CHEMBL:CHEMBL190474 ? unclear Mechanistic
CHEMBL:CHEMBL91163 ? unclear Mechanistic
IMETELSTAT SODIUM inhibits Mechanistic
SODIUM METAARSENITE inhibits Mechanistic
HLA-A*0201 RESTRICTED TERT(572Y)/TERT(572) PEPTIDES VACCINE VX-001 ? unclear Mechanistic
HTERT VACCINE V934/V935 ? unclear Mechanistic
9,10-PHENANTHRENEQUINONE ? unclear Mechanistic
CHEMBL:CHEMBL89977 ? unclear Mechanistic
IODINE I-131 ? unclear Mechanistic
TERTOMOTIDE ? unclear Mechanistic
HTERT-LAMP MRNA-LOADED AUTOLOGOUS DENDRITIC CELL VACCINE GRNVAC1 ? unclear Mechanistic
ANTISENSE OLIGONUCLEOTIDES ? unclear Mechanistic
TELOMESTATIN ? unclear Mechanistic
CHEMBL:CHEMBL1254091 ? unclear Mechanistic
RECOMBINANT BRAIN-DERIVED GROWTH FACTORS ? unclear Mechanistic
CHEMBL:CHEMBL89250 ? unclear Mechanistic
CHEMBL:CHEMBL481161 ? unclear Mechanistic
RALOXIFENE HYDROCHLORIDE ? unclear Mechanistic
CHEMBL:CHEMBL520095 ? unclear Mechanistic
CHEMBL:CHEMBL457246 ? unclear Mechanistic
CHEMBL:CHEMBL608862 ? unclear Mechanistic
CHEMBL:CHEMBL2336666 ? unclear Mechanistic
ISOBONDUCELLIN ? unclear Mechanistic
DENDRITIC CELL VACCINE ? unclear Mechanistic
ERIBULIN ? unclear Mechanistic
EPITALON ? unclear Mechanistic
CHEMBL:CHEMBL144757 ? unclear Mechanistic
SURATADENOTUREV ? unclear Mechanistic
RECOMBINANT VASCULAR ENDOTHELIAL GROWTH FACTOR ? unclear Mechanistic
BERBERINE ? unclear Mechanistic
CHEMBL:CHEMBL481160 ? unclear Mechanistic
CHEMBL314057 inhibits Mechanistic 7300.0 nM (IC50)
CHEMBL86984 inhibits Mechanistic 9500.0 nM (IC50)
CHEMBL262163 inhibits Mechanistic 45200.0 nM (IC50)
CHEMBL405650 inhibits Mechanistic 11100.0 nM (IC50)
CHEMBL313020 inhibits Mechanistic 10600.0 nM (IC50)

Showing top 40 of 211 agents ranked by evidence tier.

Therapeutic Agent Effect Direction Evidence Tier Potency Source
INTERFERON ALFA-2B ? unclear Mechanistic
PIOGLITAZONE HYDROCHLORIDE ? unclear Mechanistic
SILYMARIN ? unclear Mechanistic
RITUXIMAB ? unclear Mechanistic
VERAPAMIL ? unclear Mechanistic
PROTEASOME INHIBITOR ? unclear Mechanistic
FRESOLIMUMAB ? unclear Mechanistic
ATENOLOL ? unclear Mechanistic
BINTRAFUSP ALFA ~ modulates Mechanistic
NISEVOKITUG ? unclear Mechanistic
LUSPATERCEPT inhibits Mechanistic
TRETINOIN ? unclear Mechanistic
HYDROCORTISONE BUTYRATE ? unclear Mechanistic
VITAMIN E ? unclear Mechanistic
IRINOTECAN HYDROCHLORIDE ? unclear Mechanistic
INOSITOL ? unclear Mechanistic
NICOTINE POLACRILEX ? unclear Mechanistic
FENRETINIDE ? unclear Mechanistic
MELATONIN ? unclear Mechanistic
ISOTRETINOIN ? unclear Mechanistic
VACTOSERTIB ? unclear Mechanistic 12.9 nM (IC50)
TRIAMCINOLONE ? unclear Mechanistic
TAMOXIFEN ? unclear Mechanistic
ADRIAMYCIN ? unclear Mechanistic
HEPARAN SULFATE ? unclear Mechanistic
GOSSYPOL ? unclear Mechanistic
TCDD ? unclear Mechanistic
AMIFOSTINE ANHYDROUS ? unclear Mechanistic
RAMIPRIL ? unclear Mechanistic
ANTIOXIDANT ? unclear Mechanistic
SOTATERCEPT ? unclear Mechanistic
ASPIRIN ? unclear Mechanistic
GALUNISERTIB ? unclear Mechanistic
KEYHOLE LIMPET HEMOCYANIN ? unclear Mechanistic
PIRFENIDONE ? unclear Mechanistic
H2O2 ? unclear Mechanistic
THERAPEUTIC ANDROGEN ? unclear Mechanistic
SANDOSTATIN ? unclear Mechanistic
BROXURIDINE ? unclear Mechanistic
DISITERTIDE ? unclear Mechanistic

Showing top 40 of 62 agents ranked by evidence tier.


Clinical Trials

Recruiting and recently completed trials from ClinicalTrials.gov. Data retrieved 2026-07-03.

NCT00001456 Phase: N/A Started: 1995-11-06

Hermansky-Pudlak Syndrome (HPS) is an inherited disease which results in decreased pigmentation (oculocutaneous albinism), bleeding problems due to a platelet abnormality (platelet storage pool defect), and storage of an abnormal fat-protein compound (lysosomal accumulation of ceroid lipofuscin). T...

NCT07482917 Phase: NA Started: 2026-03-09

The OPTIMIZE-ILD-1 trial is a prospective, randomized, open-label clinical trial designed to evaluate the impact of a coordinated diagnostic pathway on patients with suspected interstitial lung disease (ILD). In routine clinical practice, diagnostic workflows for ILD are frequently fragmented, invol...

NCT06821464 Phase: PHASE1 Started: 2025-02-17

The study is being conducted to evaluate the safety and pharmacokinetics of HRS-9813 after multiple oral administration in healthy subjects.

NCT00084305 Phase: N/A Started: 2004-06-09

The etiology of pulmonary fibrosis is unknown. Analyses of blood, genomic DNA, and specimens procured by bronchoscopy, lung biopsy, lung transplantation, clinically-indicated extra-pulmonary biopsies, or post-mortem examination from individuals with this disorder may contribute to our understanding ...

NCT06329401 Phase: PHASE2 Started: 2024-04-03

A randomized, double-blind, placebo-controlled clinical study to evaluate the safety and efficacy of 2 doses of inhaled pirfenidone (AP01) versus placebo on top of standard of care in participants with PPF over 52 weeks.

NCT06951217 Phase: PHASE2 Started: 2025-04-17

This is an open-label extension study for participants who were previously enrolled in and completed an Avalyn Pharma Sponsored study with an inhaled antifibrotic, such as AP01. Eligible participants will have their final dose of drug at the end of study visit from the lead-in study and first AP-LTE...

NCT06968845 Phase: PHASE2 Started: 2026-02-02

Rationale: LTI-03 is an experimental medication breathed into the lungs using an inhaler. It is being studied for the treatment of Idiopathic Pulmonary Fibrosis (IPF). IPF is a progressive, fatal lung disease caused by the death of lung cells involved in oxygen uptake and by progressive fibrosis (sc...

NCT06325696 Phase: PHASE2 Started: 2025-05-05

Background: Interstitial lung disease affects the tissues that aid the transfer of oxygen and carbon dioxide between the air and the bloodstream. The disease can cause fibrosis, a thickening and scarring of lung tissue. Fibrosis often continues getting worse, and most people with this disease die i...

NCT07284459 Phase: PHASE2 Started: 2026-01-08

This is a Ph 2, randomized, double-blind, placebo-controlled global multicenter study to evaluate the efficacy, safety, tolerability, and pharmacokinetics (PK) of PIPE-791 in participants with a diagnosis of Idiopathic Pulmonary Fibrosis (IPF) with or without background treatment.

NCT06488638 Phase: NA Started: 2026-02-13

Idiopathic pulmonary fibrosis (IPF) is a type of scarring (fibrotic) lung disease. Reduced exercise capacity is a key symptom experienced by patients. In previous research the investigators identified that an interval-based exercise programme led to significant improvements in exercise capacity (Wal...

NCT05964335 Phase: PHASE2 Started: 2024-02-06

The main purpose of the study is to evaluate the effect of NAL ER on 24-hour cough frequency using objective digital cough monitoring and to assess safety and tolerability of NAL ER.

NCT03312400 Phase: PHASE2 Started: 2018-02-08

Background: DNA is a structure in the body. It contains data about how the body develops and works. Telomeres are found on the end of chromosomes in DNA. Some people with short telomeres or other gene changes can develop diseases of the bone marrow, lung, and liver. Researchers want to see if low d...

NCT07652658 Phase: PHASE2 Started: 2026-06-11

This Phase IIb study aims to evaluate the efficacy, safety, and tolerability of 3 doses of AZD8965 treatment compared to placebo in participants with IPF, including those on antifibrotic therapy (nintedanib, pirfenidone, nerandomilast), either alone or in combination, or in those not on antifibrotic...

NCT07036523 Phase: PHASE2 Started: 2025-11-13

This study is open to adults who are at least 40 years old and have idiopathic pulmonary fibrosis (IPF). People can participate in the study if they have a forced vital capacity (FVC) greater than or equal to 45% of the predicted value and fibrosis of 20% or more confirmed by a high-resolution compu...

NCT07201922 Phase: PHASE3 Started: 2026-02-10

This study is open to people aged 40 years or older who have at least 1 family member with pulmonary fibrosis. Pulmonary fibrosis is a condition where lung tissue becomes scarred, making it harder to breathe. People can join if a lung scan shows early changes in the lung, called interstitial lung ab...


Agents Found By Disease-Level Search

Searched directly from “Interstitial Lung Disease / Pulmonary Fibrosis” via ClinicalTrials.gov interventions and Open Targets' disease→drug data — independent of the 7-gene target panel above. This surfaces agents whose mechanism doesn't route through a curated gene (combination therapies, standard-of-care drugs, targets outside the panel).

Not yet run. python -m biomarker_pipeline.run_disease_agent_discovery --disease "..."

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