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Scleroderma

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:1221
Name:Scleroderma
Associated with:0 target
4 immuno-relevant ligands
Synonyms
systemic sclerosis
Description
A fibrosing autoimmune disease affecting collagen, characterised by abnormal growth of connective tissue in the skin and internal organs.
Database Links
Disease Ontology: DOID:419

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

No target related data available for Scleroderma

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
CXCL4 4
Immuno Disease Comments: CXCL4 levels correlate with skin and lung fibrosis in patienst with scleroderma, and is he only chemokine whose level is predictedive of the risk and progression of scleroderma.
Bioactivity Comments: CXCL4 appears to be the predominant ligand for CXCR3B, in comparison to the CXCR3 ligands CXCL9, -10 and -11. CXCL4 displaces binding of 125I-CXCL10 to CXCR3B with an IC50 of 7.46 nM, and displaces 125I-CXCL4 binding with an IC50 of 1.85 nM [2]. | View biological activity
CXCL6 3
Immuno Disease Comments: Serum CXCL6 levels are reported to positively correlate with the severity of dermal and pulmonary fibrosis and vascular symptoms in patients with systemic sclerosis (scleroderma).
iloprost
Immuno Disease Comments: Scleroderma is one of the approved indications for iloprost.
Clinical Use: Iloprost is used to treat pulmonary arterial hypertension, scleroderma, Raynaud's phenomenon and ischemia. Aurlumyn® branded iloprost was FDA approved in February 2024, as a treatment to reduce the risk of digit amputations resulting from severe frostbite. | View clinical data
anifrolumab
Immuno Disease Comments: Clinical candidate for scleroderma.
Clinical Use: Anifrolumab was progressed to clinical development for SLE and for scleroderma. First approval was granted by the FDA in August 2021, for the indication of moderate to severe systemic lupus erythematosus (SLE) in patients who are receiving standard therapy. | View clinical data
Bioactivity Comments: Anifrolumab inhibits the antiproliferative effect of IFNω on Daudi cells with an EC50 value of 0.3nM [1]. | View biological activity

References

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1. Cardarelli JM, Witte A, Srinivasan M. (2010) Interferon alpha receptor I antibodies and their use. Patent number: US7662381. Assignee: Medarex, Inc.. Priority date: 21/06/2004. Publication date: 16/02/2010.

2. Lasagni L, Francalanci M, Annunziato F, Lazzeri E, Giannini S, Cosmi L, Sagrinati C, Mazzinghi B, Orlando C, Maggi E et al.. (2003) An alternatively spliced variant of CXCR3 mediates the inhibition of endothelial cell growth induced by IP-10, Mig, and I-TAC, and acts as functional receptor for platelet factor 4. J Exp Med, 197 (11): 1537-49. [PMID:12782716]

3. Taniguchi T, Asano Y, Nakamura K, Yamashita T, Saigusa R, Ichimura Y, Takahashi T, Toyama T, Yoshizaki A, Sato S. (2017) Fli1 Deficiency Induces CXCL6 Expression in Dermal Fibroblasts and Endothelial Cells, Contributing to the Development of Fibrosis and Vasculopathy in Systemic Sclerosis. J Rheumatol, 44 (8): 1198-1205. [PMID:28507181]

4. van Bon L, Affandi AJ, Broen J, Christmann RB, Marijnissen RJ, Stawski L, Farina GA, Stifano G, Mathes AL, Cossu M et al.. (2014) Proteome-wide analysis and CXCL4 as a biomarker in systemic sclerosis. N Engl J Med, 370 (5): 433-43. [PMID:24350901]