TABLE 2

Current phase II–III trials in idiopathic pulmonary fibrosis (IPF)

	Mechanism of action	Clinical trial identifier	Study description	Primary outcome measures	Phase of development	Treatment duration
PRM-151	Recombinant form of human SAP	NCT02550873	Randomised, double-blind, placebo controlled	Change from baseline in FVC % pred	II	28 weeks
Simtuzumab	Anti-LOX antibody	NCT01769196	Randomised, double-blind, placebo-controlled	The effect of simtuzumab (GS-6624) on progression-free survival	II	148 weeks
Tipelukast	Leukotriene antagonists	NCT02503657	Randomised, double-blind, placebo controlled	Change from baseline FVC at 26 weeks	II	26 weeks
Tralokinumab	Anti IL-13 antibody	NCT01629667	Randomised dose-ranging	Change from baseline FVC % pred at week 52	II	52 weeks
SAR156597	Anti IL-4 and IL-13 antibody	NCT01529853	Randomised, double-blind, placebo-controlled	Safety/tolerability: number of participants with adverse events	II	6 weeks
Lebrikizumab	Anti IL-13 antibody	NCT01872689	Randomised, double-blind, placebo-controlled	Annualised rate of decrease in FVC % pred over 52 weeks	II	52 weeks
BG00011	Anti-integrin antibody	NCT03573505	Randomised, double-blind, placebo-controlled	Yearly rate of change in FVC	II	52 weeks
Pamrevlumab (FG-3019)	Anti-CTGF antibody	NCT01890265	Randomised, double-blind, placebo-controlled	Change from baseline in FVC % pred at week 48	II	48 weeks
PBI-4050	GPR84 antagonist/GPR40 agonist	NCT02538536	Open-label, single arm, exploratory, observational study	Number of subjects with abnormal laboratory values and/or adverse events that are related to treatment	II	20 weeks
KD025	Selective inhibitor of ROCK2	NCT02688647	Randomised, phase 2, open-label	Change in FVC in baseline to 24 weeks	II	24 weeks
CC-90001	Kinase inhibitor targeting JNKs	NCT03142191	Randomised, double-blind, placebo-controlled	Percentage point change in FVC % pred	II	24 weeks
GLPG1690	Autotaxin-LPA inhibitor	NCT02738801	Randomised, double-blind, parallel group, placebo-controlled	Safety, tolerability, pharmacokinetic and pharmacodynamic properties of GLPG1690	II	12 weeks
Omipalisib/GSK2126458	Inhibitor of PI3K/Akt pathway	NCT01725139	Randomised, double-blind, placebo-controlled	To explore a number of doses of GSK2126458 for engagement of pharmacology after short-term dosing	I	7–10 days
Sirolimus	mTOR inhibitor	NCT01462006	Double-blind placebo-controlled pilot study	Change in peripheral blood concentration of CXCR4⁺ fibrocytes; number of subjects with drug side-effects	NA	22 weeks
Rituximab	Antibody targeting CD20	NCT01969409	Randomised, double-blind, placebo-controlled	Titres of anti-HEp-2 autoantibodies, by indirect immunofluorescence assays over 9 months	II	36 weeks
Co-trimoxazole or doxycycline	Antimicrobial drugs	NCT02759120	Randomised, un-blinded, phase III	Time to first non-elective, respiratory hospitalisation or all-cause mortality	III	9 months

SAP: serum amyloid P; FVC: forced vital capacity; LOX: lysyl oxidase; IL: interleukin; CTGF: connective tissue growth factor; GPR: G protein-coupled receptor; ROCK: ρ-associated coiled-coil containing protein kinase; JNK: Jun N-terminal kinase; LPA: lysophosphatidic acid; PI3K/Akt: phosphoinositide 3-kinase/protein kinase B. mTOR: mammalian target of rapamycin; CXCR: C-X-C chemokine receptor; HEp: human epithelial cell.