Table 5.
Clinical trials currently evaluating pirfenidone therapy for fibrotic lung diseasea
| Disease entity | Investigational drug(s) | Primary end point | Current status | Study duration | Comments |
|---|---|---|---|---|---|
| IPF (NCT02598193) | Pirfenidone plus nintedanib (may have synergistic effects) | Percentage of patients completing 24 weeks of therapy | Phase IV Recruiting | 24 weeks | Safety study |
| IPF (NCT02579603) | Pharmacology of nintedanib when given with pirfenidone | Tolerability to week 12 | Phase IV Recruiting | 12 weeks | Safety, tolerability, and nintedanib PK |
| IPF (NCT02648048) | Pirfenidone plus vismodegib (synergistic antifibrotic effects) | Adverse events | Phase I Recruiting | 24 weeks | Vismodegib is a hedgehog signaling pathway inhibitor |
| IPF (NCT02707640) | Pirfenidone plus NAC (synergistic effect) | Percentage with dose reductions and adverse events | Phase II Completed | 24 weeks | European |
| IPF (NCT01872689) | Lebrikizumab plus pirfenidone | Absolute change in % predicted FVC | Phase II Ongoing | 52 weeks | |
| IPF (NCT02009293) | Pirfenidone | Change in cough frequency | Recruiting | 12 weeks | Europe |
| Hermansky–Pudlak syndrome (NCT00001596) | Pirfenidone | ΔFVC | Phase II Ongoing | 36 months | US – National Institutes of Health |
| SSc-ILD (LOTUSS) (NCT01933334) | Pirfenidone plus nintedanib | Treatment-emergent adverse events | Phase II Completed | 16 weeks | See reference 61 Multinational |
| Hypersensitivity pneumonitis (NCT02496182) | Pirfenidone | ΔFVC | Phase II/III Recruiting | 52 weeks | Mexico |
| RA-ILD (NCT02808871) | Pirfenidone | Composite (≥10% decline in FVC or death) | Phase II Not yet recruiting | 52 weeks | US study |
| Radiation lung injury (NCT02296281) | Pirfenidone | Change in lung injury classification | Phase II Not yet recruiting | 36 weeks | China |
| Amyopathic dermatomyositis with progressive ILD (NCT02821689) | Pirfenidone | 12-month survival | Phase IV Not yet recruiting | 12 months | RenJi Hospital, China |
| BOS (NCT02262299) | Pirfenidone | ΔFEV1 >6 months | Phase II/III | 6 months | European |
Note:
a
Data from www.ClinicalTrials.gov.
Abbreviations: BOS, bronchiolitis obliterans syndrome; FEV1, forced expiratory volume in 1 second; FVC, forced vital capacity; ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis; RA, rheumatoid arthritis; SSc, systemic sclerosis.