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Idiopathic pulmonary fibrosis

GtoPdb Disease Summaries

This section gives an overview of the disease, and where available shows the following:

  • Synonyms: Shows known synonyms for the disease.
  • Description: Gives a basic description/definition of the disease.
  • Database Link: External links to the same disease at the Disease Ontology, OMIM or Orphanet sites.
  • Immunopharmacology comments: General comments about the target's role in immunopharmacology, provided by GtoImmuPdb curators.
  • Associated with: Counts are displayed for the total targets the disease is associated with in GtoPdb. The counts of targets and ligands of immunological relevance associated to the disease are also shown.

More information can be found in the help pages.

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

Targets

GtoPdb Disease Summaries - Targets

Click on the target name to link to its detailed view page

Where available, information is display on the role of the target in the disease; drugs which target the disease and their therapeutic use and side-effects.

If there is mutation data curated in GtoPdb this is indicated, with a link back to the appropriate section on the target detailed view page

Immuno ligand interactions - If available, a table of immuno-relevant ligands is shown. These ligands have been curated as having an association to the disease and possess interaction data with the target in GtoPdb. The approval status of the ligand is shown, along with curator comments and an indication of whether the target is considered the primary target of the ligand.

More information can be found in the help pages.

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Key to terms and symbols
dipeptidyl peptidase 9
References:  4

Ligands

GtoPdb Disease Summaries - Ligands

Click ligand name to view ligand summary page

  • Approved: If the ligand is an approved drug this is indicated, along with approval bodies.
  • Immuno: Immuno icon indicates the ligand is immuno-relevant

Click the arrow in the final column to expand comments

  • Immuno Disease Comments: Curatorial comments specifically added as part of GtoImmuPdb. They give more information on the association between the ligand and disease in the context of immunopharmacology.
  • Clinical Use: General clinical comments relating to the ligand and may not necessarily be specific to the disease in question. With hyperlink to more details on the ligand summary pages.
  • Bioactivty Comments: Curatorial comments specifically about the compounds biological activity - with hyperlink to more details on the ligand summary pages.

More information can be found in the help pages.

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Key to terms and symbols Click ligand name to view ligand summary Click column headers to sort
Ligand References Clinical and Disease comments
IL-13 1
Immuno Disease Comments: Failed clinical target for IPF and asthma.
pirfenidone
Immuno Disease Comments: Approved drug for IPF.
Clinical Use: Approved to treat idiopathic pulmonary fibrosis (IPF) [7]. | View clinical data
Bioactivity Comments: As the precise MMOA of this drug is not fully understood, we are unable to define a primary drug target. | View biological activity
lebrikizumab
Immuno Disease Comments: Repurposed clinical candidate for IPF (Phase 2).
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 ClinicalTrails.gov's listing of current lebrikizumab trials. Lebrikizumab was most recently redirected for inflammatory skin conditions. The EMA approved lebrikizumab for the treatment of moderate-to-severe atopic dermatitis in November 2023 [6], with FDA approval for this indication following in September 2024. | View clinical data
Bioactivity Comments: In humans lebrikizumab has systemic effects on markers of Th2 inflammation. Lebrikizumab treatment reduces serum immunoglobulin E (IgE) and interleukin-13 and -17 levels by approximately 25% [9].
The development of lebrikizumab is covered by patent US7674459, in which it appears to be the monoclonal designated as 228B/C-1 [5]. No binding constant is provided in this patent, although a graph representing binding of 228B/C-1 to IL-13 in an ELISA suggests a half max value of approximately 0.1nM. | View biological activity
belumosudil
Immuno Disease Comments: Phase 2 clinical candidate for IPF (NCT02688647).
Clinical Use: Belumosudil (KD025) was evaluated in clinical studies for immunosuppressive, anti-inflammatory and anti-fibrotic efficacy. The FDA approved belumosudil (Rezurock®) in July 2021, as a treatment for chronic graft-versus-host disease that has failed to be controlled by at least two prior lines of systemic therapy. | View clinical data
ziritaxestat
Immuno Disease Comments: Clinical candidate for IPF (Phase 2 NCT02738801)
Clinical Use: GLPG1690 was progressed to Phase 3 evaluation in IPF [8] and has completed a Phase 2 study in patients with scleroderma. Both of these programmes were discontinued in early 2021, due to lack of acceptable clinical benefit at intermin data analysis in the IPF study. | View clinical data
Bioactivity Comments: GLPG1690 produces a sustained decrease in plasma LPA levels in vivo and this results in reduced extracellular matrix deposition in the lung tissue of mice with bleomycin-induced pulmonary fibrosis [3] | View biological activity
setogepram
Immuno Disease Comments: Phase 2 clinical candidate for IPF- see NCT02538536
Clinical Use: PBI-4050 reached Phase 2 clinical evaluation in a variety of inflammatory and fibrotic conditions. Click here to link to the complete list of PBI-4050 trials registered with ClinicalTrials.gov. Liminal BioSciences, who were developing the compound, decided not to progress it for IPF or hypertriglyceridemia following unsatisfactory interim pharmacokinetic results from their multiple-ascending dose study. | View clinical data

References

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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. fiercebiotech.com, https://www.fiercebiotech.com/biotech/sanofi-ditches-il4-il13-antibody-drug-lung-scarring-disease

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. Desroy N, Housseman C, Bock X, Joncour A, Bienvenu N, Cherel L, Labeguere V, Rondet E, Peixoto C, Grassot JM et al.. (2017) Discovery of 2-[[2-Ethyl-6-[4-[2-(3-hydroxyazetidin-1-yl)-2-oxoethyl]piperazin-1-yl]-8-methylimidazo[1,2-a]pyridin-3-yl]methylamino]-4-(4-fluorophenyl)thiazole-5-carbonitrile (GLPG1690), a First-in-Class Autotaxin Inhibitor Undergoing Clinical Evaluation for the Treatment of Idiopathic Pulmonary Fibrosis. J Med Chem, 60 (9): 3580-3590. [PMID:28414242]

4. 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]

5. Fung SC, Moyle M. (2010) Treatment of Interleukin13 dependent neoplastic disorders. Patent number: US7674459. Assignee: Genentech, Inc.. Priority date: 23/12/2003. Publication date: 09/03/2010.

6. Keam SJ. (2024) Lebrikizumab: First Approval. Drugs, 84 (3): 347-353. [PMID:38388870]

7. 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]

8. Maher TM, Kreuter M, Lederer DJ, Brown KK, Wuyts W, Verbruggen N, Stutvoet S, Fieuw A, Ford P, Abi-Saab W et al.. (2019) Rationale, design and objectives of two phase III, randomised, placebo-controlled studies of GLPG1690, a novel autotaxin inhibitor, in idiopathic pulmonary fibrosis (ISABELA 1 and 2). BMJ Open Respir Res, 6 (1): e000422. [PMID:31179008]

9. Scheerens H, Arron JR, Zheng Y, Putnam WS, Erickson RW, Choy DF, Harris JM, Lee J, Jarjour NN, Matthews JG. (2014) The effects of lebrikizumab in patients with mild asthma following whole lung allergen challenge. Clin Exp Allergy, 44 (1): 38-46. [PMID:24131304]