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Hypereosinophilic syndrome, idiopathic; HES

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


platelet derived growth factor receptor alpha


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: FDA and EMA orphan therapy for hypereosinophilic syndrome and eosinophilic granulomatosis with polyangiitis (also known as Churg-Strauss syndrome or allergic granulomatosis).
Clinical Use: Mepolizumab is approved for the treatment of severe asthma 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.
Click here to link to's full list of mepolizumab trials, including ongoing trials in additional eosinophilic conditions. | View clinical data
Bioactivity Comments: In monkey studies mepolizumab decreased eosinophil count with an IC50 of 1.43 μg/ml, and exhibited a half-life of ~14.5 days [3]. | View biological activity


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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. Zia-Amirhosseini P, Minthorn E, Benincosa LJ, Hart TK, Hottenstein CS, Tobia LA, Davis CB. (1999) Pharmacokinetics and pharmacodynamics of SB-240563, a humanized monoclonal antibody directed to human interleukin-5, in monkeys. J Pharmacol Exp Ther, 291 (3): 1060-7. [PMID:10565825]