Table 1 Therapeutics in latest clinical trials with primary end points specific to ECM stiffness.
TargetDrug name
(trade name/company)		CategoryHighest
completed trial		Current trials relevant
to ECM stiffness		References
miR-29MRG-201
(miRagen Therapeutics Inc.)		MicroRNAPhase 1None(26)
NCT02603224
CTGFFG-3019 (Fibrogen)Monoclonal antibodyPhase 2Phase 2(33, 34)
NCT01890265
NCT01262001
TGFβ signalingPirfenidone
(Esbriet/Genentech)		Small molecule,
mechanism unknown		Approved:
Idiopathic lung fibrosis		Phase 1–3(36)
NCT01872689
NCT02552849
NCT02408744
NCT03068234
NCT01872689
TGFβGC1008
(Fresolimumab/Genzyme)		Monoclonal antibodyPhase 2Phase 1–2(37)
NCT01401062
NCT02581787
NCT01665391
VEGF, FGF, and
PDGF receptors		Nintedanib
(Ofev/Boehringer Ingelheim)		Small molecule, tyrosine
kinase inhibitor		Approved:
Idiopathic lung fibrosis		Phase 1–3NCT01284322
LOXL2GS-6624
(Simtuzumab/Gilead)		Monoclonal antibodyPhase 2
No efficacy in fibrosis
or cancer		None(56–58)
NCT01472198
NCT01759511
NCT 01769196
LOXTetrathiomolybdateCopper chelatorPhase 3
(Wilson’s disease)		Phase 2(60)
NCT00805805
NCT00195091
NCT01837329
αvβ6BG00011/STX-100 (Biogen)Monoclonal antibodyPhase 2None(89)
NCT01371305
FAKVS-6063/PF-04554878
(Defactinib/Verastem)		Small moleculePhase 2Phase 1–2(117)
NCT02943317
NCT02758587
NCT02546531
NCT02465060
FAKGSK-2256098
(GlaxoSmithKline)		Small moleculePhase 1Phase 1–2(117)
NCT02551653
NCT02428270
NCT02523014
NF-κBBortezomib
(Velcade/Takeda)		Small moleculeApproved:
Multiple myeloma		Phase 2(138, 139)
NCT02370693