Table 2.
Emerging antifibrotic therapies for pulmonary fibrosis
| Drug | Mechanism of action | Target population | Development status | Notes |
|---|---|---|---|---|
| BI 1015550 | Selective phosphodiesterase 4B (PDE4B) inhibitor | IPF and PPF | Phase III (FIBRONEER-ILD [100]) | Shown to preserve FVC in IPF patients, may reduce inflammation and fibrosis |
| BMS-986278 [101] | Lysophosphatidic acid receptor 1 antagonist | IPF and PF-ILD | Phase II | Targets fibroblast activation and migration; potential to slow progression |
| Treprostinil, inhaled | Prostacyclin analogue | PPF | Phase III (TETON study [102]) | Primary endpoint is change in FVC at week 52 |
| Pamrevlumab | Monoclonal antibody against CTGF | IPF | Phase III (ZEPHYRUS-1 [103]) | No significant change in FVC vs. placebo in IPF patients |
| PRM-151 (ziltivekimab) | Recombinant human pentraxin-2 protein (fibrosis modulator) | IPF | Phase III (STARSCAPE [104]) | No benefit patients with IPF vs. placebo in FVC decline |
CTGF connective tissue growth factor, FVC forced vital capacity, IPF idiopathic pulmonary fibrosis, PF-ILD progressive fibrotic ILD, PPF progressive pulmonary fibrosis