|
| Drug name | Target | Mechanism of action | Trial ID/reference |
|
| Treatments against immune mediators |
|
Anifrolumab
(MEDI546) | Directed against subunit 1 of type I interferon receptor | Downregulation of T-cell activation | NCT00930683 |
|
| Inebilizumab (MEDI551) | Anti-CD19 | Depletion of B cells through enhanced antibody-dependent cellular cytotoxicity | NCT00946699 |
|
Sifalimumab
Rontalizumab | Anti-IFNα | Specific for IFNα blocking; doesn’t neutralize other type I IFNs | NCT01283139 |
| NCT00541749 |
|
| MEDI7734 | Anti-ILT7 | Temporary depletion of plasmacytoid dendritic cells | NCT02780674 |
|
| Rituximab | Anti-CD20 | Targets CD20 expressed from pre-B cell stage to the pre-plasma cell stage | Giuggioli D et al. [10] |
|
| Basiliximab | Anti-IL-2Rα | Directed against the α chain (CD25) of the IL-2 receptor | Becker MO et al. [11] |
|
| Efalizumab | LFA1/ICAM-1 | Interaction between LFA-1 and ICAM-1 is blocked, preventing T-cell’s activation | Zimmerman T et al. [12] |
|
| Abatacept | CTLA4 | Inhibits T-cell activation by selectively modulating costimulation (binds to CD80 or CD86 on cell surface) | Elhai M et al. [13] |
|
| AIMSPRO (®) | αMSH, IL10, CCL2 | Modulates serum levels of relevant cytokines | Quillinan NP et al. [14] |
|
| Tocilizumab | IL-6R | Regulatory effect in the balance between Th17 and Tregs | NCT01532869 |
|
| AM095 and SAR100842 | LPA1 | Targets specific G-protein-coupled receptors | NCT01651143 |
|
| TAK242 | TLR4 | Prevents Th1 and Th17 cytokines production by inhibiting TLR4 stimulation | Bhattacharyya S et al. [15] |
|
| Treatments against Fibrosis |
|
| Imatinib | PDGF, TGF-β | Blocks both PDGF and TGF-β signalling pathways | Iwamoto N et al. [16] |
|
Dasatinib,
Nilotinib | c-Abl, PDGF | Second-generation TKIs with higher affinity to Bcr-Abl | Akhmetshina A et al. [17] |
|
| Metelimumab (CAT-192) | TGFβ1 | Specifically counteracts the TGFβ1 isoform | Denton CP et al. [18]
|
|
| Fresolimumab (GC-1008) | TGFβ1,-β2,-β3 | Targets all isoforms of TGFβ | NCT01284322 |
|
| FG-3019 | CCN2 | Anti-CTGF, reduces the number of CD45-positive cells | Brenner MC et al. [19] |
|
| P144 | TGFβ1 | Blocks the interaction between TGFβ1 and TGFβ1 type III receptor | NCT00574613,
Postlethwaite AE et al. [20] |
|
| Anti-Integrin V6 (Abituzumab) | TGFβ | Inhibits binding to αv heterodimers, preventing TGF-β activation | NCT02745145,
Katsumoto et al. [17],
Henderson NC et al. [21] |
|
| Pirfenidone | TNFα, IL1β, TGFβ | Blocks TGF-β-stimulated collagen synthesis | Udwadia ZF et al. [22]
Khanna D et al. [23] |
|
Nintedanib
(BIBF 1120) | VEGF, PDGF, FGF | TKI-targeting FGF, PDGF, and VEGF receptors, as well as Src-family tyrosine kinases | NCT02597933,
Distler et al. [24] |
|
| Treatments against vascular alterations |
|
Bosentan,
Macitentan | ETA/ETB receptor | Blocks both ETA and ETB endothelin receptors that mediate the effects of ET-1 | NCT00077584,
Matucci-Cerinic M et al. [25]
NCT00660179,
Metha S et al. [26] |
|
| Ambrisentan | ETA receptor | Blocks the action of endothelin-1 at the ETA receptor | NCT00091598,
NCT01178073,
Gailè N et al. [27],
Raghu G et al. [28] |
|
| Selexipag | IP receptor | Selective IP prostacyclin receptor agonist for long-term treatment of PAH | Sitbon O et al. [29],
Denton CP et al. [30] |
|
| Riociguat | GMPc agonist | Soluble guanylate cyclase (sGC) modulator with both vasoactive and antifibrotic effects | NCT02283762,
NCT00863681 |
|
| Bardoxolone methyl | Nrf2 and NF-κB | Activation of Nrf2 and inhibition of NF-κB | NCT02036970,
NCT02657356 |
|
