Table 1.
List of the main innovative therapeutic approaches in preclinical or clinical testing for IPF treatment. Treatments explored for PF resulting from COVID-19 are also reported. Therapies that already proved to be ineffective or detrimental have not been included in this list, but can be found elsewhere [165], [207]. For each therapeutic option, the name and typology of product, the molecular target, the data supporting efficacy, and, where available, the information about clinical trials started (type, name, and/or ClinicalTrials.gov identifier) are reported.
| Name and type of the product | Target | Evidence of efficacy for IPF treatment (pre-clinical cellular, preclinical-animal, clinical) | Info about clinical trial | Clinical Application | Related references |
|---|---|---|---|---|---|
| Pamrevlumab, FG-3019 (humanized monoclonal antibody) | CTGF (connective tissue growth factor) | Clinical | Phase II completed (NCT01890265) Phase III ongoing (NCT03955146) Phase II ongoing (NCT04432298) |
IPF PF in COVID-19 |
[208] |
| Ianalumab, VAY736 (humanized monoclonal antibody) | BAFF-R (B cell activating factor receptor) | Clinical | Phase II ongoing (NCT03287414) | IPF | [121] |
| Lebrikizumab (humanized monoclonal antibody in monotherapy or in combination with pirfenidone) | Interleukin 13 (IL-13) | Clinical | Phase II ongoing (NCT01872689) | IPF | [209] |
| Meplazumab (humanized monoclonal antibody) | CD147 | Clinical | Phase II ongoing (NCT04275245) | PF in COVID-19 | [210] |
| Losartan (small molecule antagonist) | Angiotensin II type 1 receptor | Clinical | Phase II ongoing (NCT00879879) | IPF PF in COVID-19 |
[211], [212] |
| PRM-151 (human recombinant protein) | Pentraxin-2 (PTX-2) | Clinical | Phase II completed (NCT02550873) Phase III ongoing (NCT04552899) |
IPF | [125] |
| IW001 (oral immunotherapy) | Col(V) collagen autoantibodies | Clinical | Phase I completed (NCT01199887) | IPF | [213] |
| Brazilian green propolis (natural compound) | Various targets | Clinical | Phase II completed (NCT04480593) | PF in COVID-19 | [214] |
| GB0139 (other name TD139) (small molecule inhibitor) | Galectin-3 | Clinical | Phase I-IIa completed Phase IIb ongoing (NCT03832946) |
IPF | [215] |
| CC-930 and CC-90001 (small molecule inhibitors) | JNK | Pre-clinical (animal) Clinical | Phase I completed Phase II ongoing (NCT03142191) |
IPF | [216], [217] |
| BMS-986278 (small molecule inhibitor) | LPA1 | Pre-clinical (cellular, and animal) Clinical | n.d. Phase II ongoing (NCT04308681) |
IPF | [218] |
| GLPG1690 (small molecule inhibitor) | Autotaxin (ENPP2) | Clinical | Phase IIa completed (NCT02738801) | IPF | [219] |
| Saracatinib (small molecule inhibitor) | Src tyrosine kinase | Clinical | Phase Ib/IIa ongoing (NCT04598919) | IPF | [220] |
| Talagedib, ENV-101 (small molecule inhibitor) | Hedgehog pathway | Clinical | Phase II ongoing (NCT04968574) | IPF | n.d. |
| ORIN1001 (small molecule inhibitor) | IRE1α/XBP1 | Clinical | Phase Ib ongoing (NCT04643769) | IPF | n.d. |
| Colchicine (small molecule inhibitor) | Inflammasome machinery | Clinical | Phase 3, randomised, double-blind, completed Phase 4, ongoing (NCT04818489) |
PF in COVID-19 | [221] |
| MSC (mesenchymal stem cell) therapy | ATII-cell intratracheal transplantation | Clinical | phase Ib. | Moderate/Progressive IPF | [222] |
| Bone-marrow MSC | intravenous infusion | Clinical | Phase I/IIa ongoing (NCT02594839) | IPF | [129] |
| Lung Spheroid Stem Cells (LSCs) | intravenous infusion | Clinical | Phase I ongoing (NCT04262167) | IPF | n.d. |
| siRNA TRK-250 | TGF-β1 | Clinical | Phase I ongoing (NCT03727802) | IPF | [223] |
| siRNA nanoparticle and prostaglandin2 | MMP3, CCL12, and HIFα mRNAs | Pre-clinical (animal) | n.d. | IPF | [224] |
| siRNA liposome | SART1 | Pre-clinical (animal) | n.d. | IPF | [225] |