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🗺️ Disease Atlas · Respiratory or thoracic disease

pulmonary fibrosis

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).

🔔 Follow weekly
🧬4287genes / proteins with evidence
💊56drugs and clinical candidates
🧪12+recruiting trials worldwide
✅2approved
📈5phase 3
🫁

Understand pulmonary fibrosis

Lungs and breathing · Airways that narrow and swell
🎓 Learn more

What it is

Causes of pulmonary fibrosis include environmental pollutants, some medicines, some connective tissue diseases, and interstitial lung disease. Interstitial lung disease is the name for a large group of diseases that inflame or scar the lungs. In most cases, the cause cannot be found. This is called idiopathic pulmonary fibrosis.

Symptoms include:

Shortness of breath

From MedlinePlus: Pulmonary Fibrosis (US National Library of Medicine) ↗

🫁 Lungs and breathing: Chronic lung diseases such as asthma and chronic obstructive pulmonary disease (COPD) damage or inflame the airways and lungs, so breathing becomes hard.

❓ Why it happens

  • Smoking, air pollution, smoke from cooking with solid fuels, dust and chemicals at work, allergies, infections and genes.

🫀 What it does to the body

  • The airways swell, tighten and fill with mucus, so less air gets in and out.
  • In COPD the tiny air sacs are slowly destroyed, and the damage does not heal.

🧱 Why it is hard to cure

  • Damage in COPD is permanent, and pollution is hard to avoid.
  • Many people are diagnosed late, or not at all.

🚀 What research is trying now

  • Biologic medicines for severe asthma, and new ones being tested in COPD.
  • Better inhaled medicines, and research into helping lung tissue repair itself.

General information about lungs and breathing, reviewed by people; it does not describe any one person. Below: the evidence for pulmonary fibrosis itself.

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AI briefing

Where the science on pulmonary fibrosis stands, from the evidence on this page

AI reads the targets, drugs, trials and papers below and writes where the science stands — every claim cited. It is shared with everyone who opens pulmonary fibrosis.

🎯

Top targets

Genes and proteins ranked by Open Targets evidence (0-1)
TERT telomerase reverse transcriptasePROTACantibodyothersmall molecule🧊 AlphaFold structure ↗ 0.86
RTEL1 regulator of telomere elongation helicase 1PROTAC🧊 AlphaFold structure ↗ 0.84
PARN poly(A)-specific ribonucleasePROTACsmall molecule🧊 AlphaFold structure ↗ 0.80
SFTPA2 surfactant protein A2antibody🧊 AlphaFold structure ↗ 0.74
NAF1 nuclear assembly factor 1 ribonucleoproteinPROTAC🧊 AlphaFold structure ↗ 0.73
PDGFRB platelet derived growth factor receptor betaPROTACantibodyothersmall molecule🧊 AlphaFold structure ↗ 0.62
FGFR2 fibroblast growth factor receptor 2PROTACantibodyothersmall molecule🧊 AlphaFold structure ↗ 0.61
PDGFRA platelet derived growth factor receptor alphaPROTACantibodyothersmall molecule🧊 AlphaFold structure ↗ 0.61
FGFR3 fibroblast growth factor receptor 3PROTACantibodyothersmall molecule🧊 AlphaFold structure ↗ 0.61
NR3C1 nuclear receptor subfamily 3 group C member 1PROTACothersmall molecule🧊 AlphaFold structure ↗ 0.60
FLT1 fms related receptor tyrosine kinase 1PROTACantibodyothersmall molecule🧊 AlphaFold structure ↗ 0.60
FGFR4 fibroblast growth factor receptor 4PROTACantibodyothersmall molecule🧊 AlphaFold structure ↗ 0.60
FGFR1 fibroblast growth factor receptor 1PROTACantibodyothersmall molecule🧊 AlphaFold structure ↗ 0.60
KDR kinase insert domain receptorPROTACantibodyothersmall molecule🧊 AlphaFold structure ↗ 0.60
FLT4 fms related receptor tyrosine kinase 4PROTACantibodysmall molecule🧊 AlphaFold structure ↗ 0.59

Green labels: what the target is known to be druggable by. The score combines genetics, somatic mutations, drugs, expression and literature.

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Drugs and candidates

Every medicine linked to pulmonary fibrosis, by how far it has got
Approved · 2
NintedanibPlatelet-derived growth factor receptor inhibitor; Vascular endothelial growth factor rec…Approved
Prednisolone Sodium PhosphateGlucocorticoid receptor agonistApproved
In phase 2 / 3 · 20
AdmilparantPhase 3
CyclophosphamidePhase 3
PirfenidonePhase 3
TadalafilPhosphodiesterase 5A inhibitorPhase 3
TreprostinilProstanoid IP receptor agonistPhase 3
IloprostProstanoid IP receptor agonistPhase 2/3
Show all 20
Pyruvate SodiumPhase 2/3
Air, MedicalPhase 2
Ascorbic AcidPhase 2
AvenciguatPhase 2
BevacizumabVascular endothelial growth factor A inhibitorPhase 2
Bortezomib26S proteasome inhibitorPhase 2
CarlumabC-C motif chemokine 2 inhibitorPhase 2
DanazolProgesterone receptor agonist; Androgen Receptor agonistPhase 2
DazcapistatPhase 2
DexamethasoneGlucocorticoid receptor agonistPhase 2
EndostatinPhase 2
HydrocortisoneGlucocorticoid receptor agonistPhase 2
HymecromonePhase 2
Ifetroban SodiumPhase 2
Early trials · 12
AcetylcysteinePhase 1/2
CarboplatinPhase 1/2
ErythromycinBacterial 70S ribosome inhibitorPhase 1/2
GenisteinPhase 1/2
LosartanType-1 angiotensin II receptor antagonistPhase 1/2
PaclitaxelTubulin inhibitorPhase 1/2
Show all 12
PovidonePhase 1/2
PravastatinHMG-CoA reductase inhibitorPhase 1/2
ZileutonArachidonate 5-lipoxygenase inhibitorPhase 1/2
Cc-90001Phase 1
Dabigatran EtexilateThrombin inhibitorPhase 1
Dichloroacetic AcidPhase 1
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