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Disease ID:1221
Associated with:0 target
4 immuno-relevant ligands
systemic sclerosis
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


No target related data available for Scleroderma


Key to terms and symbols Click ligand name to view ligand summary Click column headers to sort
Ligand References Clinical and Disease comments
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
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).
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. | View clinical data
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


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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]