Hypereosinophilic syndrome, idiopathic; HES

Disease ID:449
Name:Hypereosinophilic syndrome, idiopathic; HES
Associated with:1 target
1 immuno-relevant ligand
Synonyms
Hypereosinophilic syndrome of undetermined significance
Database Links
OMIM: 607685
Orphanet: ORPHA3260

Targets

platelet derived growth factor receptor alpha

Ligands

Key to terms and symbols Click ligand name to view ligand summary Click column headers to sort
Ligand References Clinical and Disease comments
mepolizumab
Immuno Disease Comments: FDA and EMA orphan therapy for hypereosinophilic syndrome and Churg-Strauss syndrome (both Phase 3)
Clinical Use: In November 2015, the US FDA approved mepolizumab for the treatment of severe asthma exacerbations in patients who are already taking other asthma medications [1-2].
In the United States and the European Union, mepolizumab can be used under orphan drug designation for the treatment of Churg-Strauss syndrome and hypereosinophilic syndrome.
To gain full marketing authorisation, mepolizumab is being evaluated in clinical trials in patients with eosinophilic COPD (Phase III), hypereosinophilic syndrome (HES; Phase III) and for eosinophilic granulomatosis with polyangiitis (EGPA or Churg-Strauss Syndrome; Phase III). Click here to view ClinicalTrials.gov's listing of current mepolizumab trials. | View clinical data
Bioactivity Comments: Smith et al (2011) cite an IC50 of 0.94nM for mepolizumab-induced inhibition of IL-5 binding to its receptor [3], although the citation for this data is a GlaxoSmithKline investigators’ brochure for mepolizumab, so is not peer reviewed or easily accessed. | View biological activity

References

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1. Bel EH, Wenzel SE, Thompson PJ, Prazma CM, Keene ON, Yancey SW, Ortega HG, Pavord ID, SIRIUS Investigators. (2014) Oral glucocorticoid-sparing effect of mepolizumab in eosinophilic asthma. N. Engl. J. Med., 371 (13): 1189-97. [PMID:25199060]

2. Ortega HG, Liu MC, Pavord ID, Brusselle GG, FitzGerald JM, Chetta A, Humbert M, Katz LE, Keene ON, Yancey SW et al.. (2014) Mepolizumab treatment in patients with severe eosinophilic asthma. N. Engl. J. Med., 371 (13): 1198-207. [PMID:25199059]

3. Smith DA, Minthorn EA, Beerahee M. (2011) Pharmacokinetics and pharmacodynamics of mepolizumab, an anti-interleukin-5 monoclonal antibody. Clin Pharmacokinet, 50 (4): 215-27. [PMID:21348536]