PF-Atlas › IPF Treatments
Pulmonary Fibrosis Treatment Options
Idiopathic pulmonary fibrosis has two FDA-approved antifibrotic drugs, pirfenidone and nintedanib, that slow the decline in lung function. A new PDE4B inhibitor was approved in 2025, and a broad Phase III pipeline is in trials. This page maps every treatment PF-Atlas tracks, tiered by evidence and linked to its source. It does not recommend any treatment.
How PF-Atlas grades evidence
Clinically established. FDA approved or positive Phase III.
Clinical research. In human clinical trials (Phase I to III).
Research model. Computational or preclinical hypothesis, not clinically validated.
Established antifibrotic therapy
The two approved antifibrotics remain the standard of care for most patients. Both slow the rate of forced vital capacity (FVC) decline rather than reverse fibrosis.
| Treatment | Mechanism | Evidence | Developer |
|---|---|---|---|
| 1Nerandomilast | Selective PDE4B inhibitor, elevates cAMP, suppresses pro-inflammatory cytokines and fibroblast activation | Clinically established Level 1 (FDA Approved) | Boehringer Ingelheim |
| 2Inhaled Treprostinil | Prostacyclin analog, vasodilation + anti-fibrotic + anti-proliferative in pulmonary vasculature and parenchyma | Clinically established Level 1 (Phase III Positive) | United Therapeutics |
| 3Admilparant | LPA1 receptor antagonist, blocks lysophosphatidic acid pro-fibrotic signaling | Clinically established Level 1 (Phase III) | Bristol-Myers Squibb |
Late-stage and research pipeline
The following agents are ranked by the PF-Atlas evidence score. Clinical status is shown for each. Agents labeled as a research model are computational or preclinical and are not clinically validated.
| Treatment | Mechanism | Evidence | Developer |
|---|---|---|---|
| 1Rentosertib | First-in-class TNIK kinase inhibitor, blocks TRAF2/NCK-interacting kinase driving fibrosis and inflammation | Clinical research Level 2 (Phase IIa) | Insilico Medicine |
| 2AI Knowledge Graph | ML system connecting mechanisms across IPF, regeneration, aging, cancer fields | Research model Computational | Insilico / Standigm / Multiple |
| 3uPAR-CAR-T Senolytic Immunotherapy | CAR-T cells targeting uPAR (urokinase plasminogen activator receptor) on senescent cells, living drug senolytic | Clinical research Level 2 (Preclinical→Phase I) | Memorial Sloan Kettering |
| 4Senolytics + WNT Agonist Combination | Sequential: D+Q clears senescent cells, RSPO3 expands AEC2 pool | Clinical research Rational Combination | UCSF / Mayo |
| 5PMFF-Net AI Diagnostic | Parallel Multi-scale Feature Fusion Network classifies ILD patterns from CT with 97.6% accuracy | Research model Computational | Multiple |
| 6Lung-on-Chip + AI Drug Screening | Emulate Bio platform screens drug combinations in hours vs months | Research model Level 4 (Platform) | Wyss Institute / Emulate |
| 7LNP-mRNA Lung-Targeted Gene Delivery | Lipid nanoparticle platform delivers therapeutic mRNA specifically to lung epithelium | Clinical research Level 2 (Platform) | UT Southwestern / UCLA |
| 8CRISPR Base Editing for AATD | Base editing corrects genetic mutations causing lung disease without DNA double-strand breaks | Clinical research Level 2 (Phase I) | Beam Therapeutics / Prime Medicine |
| 9CAR-T Anti-FAP Fibroblasts | Direct clearance of FAP+ activated myofibroblasts via chimeric antigen receptor T cells | Clinical research Level 2 (Preclinical) | Penn / Multiple |
| 10GLP-1 Agonist Anti-Fibrotic Repurposing | GLP-1 receptor agonists (semaglutide/liraglutide) show anti-fibrotic + anti-inflammatory effects across organs | Clinical research Level 2 (Repositioning) | Novo Nordisk / Eli Lilly |
| 11Dendritic Cell Immunotherapy | Engineered dendritic cells train immune system to clear fibrotic cells, promotes regulatory T-cell responses | Clinical research Level 2 (Phase I/II) | German Centers of Excellence |
| 12WNT | R-spondin 3 expands AEC2 stem cell pool via WNT pathway | Clinical research Level 3 (Phase I) | Entrega Bio / UCSF |
| 13Dasatinib + Quercetin | Senolytic, clears p16+/p21+ senescent cells, reduces SASP | Clinical research Level 2 (Phase II) | Mayo Clinic |
| 14PLN-74809 | Dual integrin inhibitor, blocks TGF-β activation at source via αvβ6/αvβ1 | Clinical research Level 2 (Phase II/III) | Pliant Therapeutics / UCSF |
| 15Rapamycin | mTOR inhibition reduces cellular senescence, autophagy dysfunction, and fibroblast proliferation in lung | Clinical research Level 2 (Repositioning) | Multiple |
| 16CD49a+ Tissue Resident Memory T-Cell Therapy | Adoptive transfer of lung-resident memory T cells reverses established fibrosis | Clinical research Level 3 (Preclinical) | Multiple |
| 17Ruxolitinib (JAK1 | Blocks SASP cytokine signaling (IL-6, IL-8) without killing senescent cells | Clinical research Level 2 (Repositioning) | Incyte / Multiple |
| 18Liver Fibrosis Reversal Protocol Crossover | Applying proven liver fibrosis reversal mechanisms to lung, stellate cell parallels to myofibroblast | Clinical research Level 2 (Cross-domain) | Icahn Sinai / Multiple |
| 19Inhaled GSK3008348 | Inhaled integrin αvβ6 inhibitor, targets TGF-β activation on AEC2s | Clinical research Level 2 (Phase II) | GSK / UCSF |
| 20OSK Partial Reprogramming of AEC2s | Inhaled LNP-mRNA OSK resets epigenetic age of AEC2s, treats root cause | Clinical research Level 3 (Preclinical) | Altos Labs / Shift Bio |
| 21Galectin-3 Inhibition | Inhaled galectin-3 inhibitor blocks fibroblast activation and TGF-β release | Clinical research Level 2 (Phase II) | Galecto Biotech |
| 22Lipofibroblast Niche Restoration | PPAR-y agonist + FGF10 restores AEC2 metabolic support niche | Clinical research Level 3 (Preclinical) | Multiple |
| 23Navitoclax | BCL-2/BCL-XL inhibitor, more potent senolytic than D+Q | Clinical research Level 2 (Phase II) | Multiple centers |
| 24ROCK Inhibitor, Inhaled Fasudil | Reduces cytoskeletal tension, reverses myofibroblast, breaks ECM stiffness loop | Clinical research Level 2 (Phase II Japan) | Multiple (Japan) |
| 25Aberrant Basaloid Cell Elimination | Target LAMB3+/KRT17+ aberrant cells, remove pro-fibrotic signal source | Clinical research Level 3 (Preclinical) | Novel |
| 26YAP | Blocks mechanosensing amplification loop, breaks stiffness-to-fibrosis cycle | Clinical research Level 3 (Preclinical) | Multiple |
| 27TERT Gene Therapy | AAV-TERT restores telomere length in AEC2s, addresses genetic root cause | Clinical research Level 3 (Preclinical) | Telomere Therapeutics |
| 28Macrophage M2 Reprogramming | LNP-mRNA IRF4/STAT6/KLF4 forces pro-regenerative macrophage state | Clinical research Level 3 (Cross-domain) | Multiple |
| 29iPSC-Derived AEC2 Transplant | Autologous iPSC-AEC2s replace depleted stem cell pool in prepared niche | Clinical research Level 3 (Preclinical) | BU/BMC |
| 30NAD+ | Restores NAD+ levels depleted in aging lungs, supports sirtuin-mediated DNA repair and mitochondrial function | Clinical research Level 3 (Phase I) | Multiple |
| 31Mocetinostat | Epigenetically unlocks fibroblasts from myofibroblast state | Clinical research Level 2 (Phase II) | Multiple |
| 32MSC-Derived Extracellular Vesicles | EV cargo (miR-29, let-7d) delivers anti-fibrotic miRNA without cell engraftment risk | Clinical research Level 3 (Phase I) | Multiple |
Frequently asked questions
What is the standard treatment for idiopathic pulmonary fibrosis?
The two FDA-approved antifibrotic drugs are pirfenidone and nintedanib. Both slow the decline in lung function. Supportive care includes supplemental oxygen, pulmonary rehabilitation, and evaluation for lung transplant in eligible patients. Treatment choice is made by a pulmonologist.
Is there a cure for pulmonary fibrosis?
There is no cure that reverses established fibrosis. Approved drugs slow progression. Several research-stage agents target repair and regeneration, but none are clinically validated for reversal.
What new pulmonary fibrosis drugs were approved recently?
A selective PDE4B inhibitor was approved in 2025 for pulmonary fibrosis. Its trial results are sourced on its treatment page. Other candidates such as LPA1 antagonists are in Phase III.
How does PF-Atlas rank treatments?
Each treatment carries an evidence tier. Clinically established means FDA approved or positive Phase III. Clinical research means it is in human trials. Research model means it is computational or preclinical. See the editorial policy for the full method.
Is this page medical advice?
No. PF-Atlas is a research atlas built by an IPF patient, not a healthcare provider. Nothing here recommends a treatment. Consult a qualified pulmonologist before any decision.
Related on PF-Atlas
Not medical advice. PF-Atlas is a research atlas built by a patient, not a healthcare provider. This page is informational and does not recommend any treatment. Always consult a qualified pulmonologist or oncologist before any treatment decision. Editorial policy and how PF-Atlas verifies data.