Idiopathic pulmonary fibrosis

Disease ID:486
Name:Idiopathic pulmonary fibrosis
Associated with:1 targets
0 immuno-relevant targets
5 immuno-relevant ligands
Database Links
Disease Ontology: DOID:0050156
Orphanet: ORPHA2032


dipeptidyl peptidase 9
References:  3


Ligand Approved Immuno References Clinical comments
IL-13 1
Immuno Disease Comments: Failed clinical target for IPF and asthma.
pirfenidone  [  ( ]
Clinical Use: Approved to treat idiopathic pulmonary fibrosis (IPF) [4].
Immuno Disease Comments: Approved drug for IPF.
Clinical Use: This antibody was originally tested as a treatment for refractory Hodgkin's lymphoma but was later repurposed as a potential asthma treatment [2]. The antibody entered Phase 2 trial for idiopathic pulmonary fibrosis (IPF) and Phase 3 trial for asthma. Business reports online indicate that Roche have discontinued lebrikizumab development in IPF following mixed results from their trials [1]. Click here to view's listing of current lebrikizumab trials.
Immuno Disease Comments: Repurposed clinical candidate for IPF (Phase 2).
Clinical Use: KD025 is in Phase 2 clinical trials evaluating its anti-inflammatory and anti-fibrotic activity.
Immuno Disease Comments: Phase 2 clinical candidate for IPF (NCT02688647).
Clinical Use: GLPG1690 is being evaluated in a Phase 2 safety, tolerability and PK/PD clinical trial in subjects with IPF (see NCT02738801).
Immuno Disease Comments: Clinical candidate for IPF (Phase 2 NCT02738801)


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1. Adams B. Sanofi ditches IL-4/IL-13 antibody drug in lung-scarring disease. Accessed on 09/02/2018. Modified on 09/02/2018.,

2. Corren J, Lemanske RF, Hanania NA, Korenblat PE, Parsey MV, Arron JR, Harris JM, Scheerens H, Wu LC, Su Z et al.. (2011) Lebrikizumab treatment in adults with asthma. N. Engl. J. Med., 365 (12): 1088-98. [PMID:21812663]

3. Fingerlin TE, Murphy E, Zhang W, Peljto AL, Brown KK, Steele MP, Loyd JE, Cosgrove GP, Lynch D, Groshong S et al.. (2013) Genome-wide association study identifies multiple susceptibility loci for pulmonary fibrosis. Nat. Genet., 45 (6): 613-20. [PMID:23583980]

4. King Jr TE, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, Gorina E, Hopkins PM, Kardatzke D, Lancaster L et al.. (2014) A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N. Engl. J. Med., 370 (22): 2083-92. [PMID:24836312]