TABLE 1.

Emerging pharmacotherapy targeting fibrogenic pathways in IPF

Name/Class	Mechanism of Action	Clinical trials	Other
Interferon-γ1b	Modulates TH1/TH2 balance; inhibits fibroblast activation	Phase 3 (completed) Phase 3 (ongoing)
Pirfenidone	Suppresses TNF-α; inhibits TGF-β; free radical scavenger	Phase 2 (completed) Phase 3 (planned)
Zileuton	Inhibits 5-lipoxygenase; Modulates eicosanoid imbalance	Phase 2 (ongoing)	Approved for asthma
Etanercept	Inhibits TNF-α receptor binding	Phase 2 (completed)
N-acetyl cysteine	Anti-oxidant; glutathione precursor	Phase 3 (completed)	Approved for acetaminophen overdose and contrast nephropathy
Tetrathiomolybdate	Inhibits angiogenesis; Inhibits TGF-β, TNF-α production; anti-oxidant	Phase 1/2 (ongoing)
Bosentan	Endothelin-1 receptor antagonist	Phase 2/3 (ongoing)	Approved for pulmonary hypertension
Monoclonal Anti-TGF-β Antibodies	Inhibits binding of TGF-β to its receptor	Phase 1/2 in scleroderma and post-trabulectomy scar prevention; initiated in IPF
TGF-β receptor kinase inhibitors	Protein kinase inhibitor; Blocks TGF-β type 1 receptor kinase activity	Planning stages for IPF
Imatinib mesylate	Protein kinase inhibitor; Inhibits c-Abl and PDGF tyrosine kinase	Phase 2 (ongoing)	Approved for CML and GIST
AG1879	Protein kinase inhibitor; Modulates myofibroblast differentiation and survival by inhibiting FAK/Src kinases and Akt.	Pre-clinical
Anti-CTGF	Monoclonal antibody against CTGF	Phase 2 (ongoing)