Disease · MONDO_0007818

hyper-IgE recurrent infection syndrome 1, autosomal dominant

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. hyper-IgE recurrent infection syndrome 1, autosomal dominant. Diseases, record MONDO_0007818. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0007818

On this page

Disease

Names and IDs as Open Targets gives them. Its IDs in MeSH, Orphanet, the Disease Ontology and the NCI Thesaurus are those Open Targets states for it.

Name: hyper-IgE recurrent infection syndrome 1, autosomal dominant; Open Targets
Open Targets ID: MONDO_0007818; Open Targets
Other names: AD hyperimmunoglobulin E syndrome, AD-HIES, Autosomal Dominant HIESs, Autosomal Recessive HIES, Autosomal Recessive HIESs, Buckley syndrome, HIES autosomal dominant, HIES, Autosomal Recessive, HIESs, Autosomal Dominant, HIESs, Autosomal Recessive, Hyper IgE Syndrome, Hyper IgE Syndrome, Autosomal Dominant, Hyper IgE Syndrome, Autosomal Recessive, Hyper Immunoglobulin E Syndrome, Autosomal Dominant, Hyper Immunoglobulin E Syndrome, Autosomal Recessive, Hyper-IgE Syndromes, Hyperimmunoglobulin E Recurrent Infection Syndrome, Hyperimmunoglobulinemia E Syndrome, Hyperimmunoglobulinemia E Syndromes, JOB syndrome, Job syndrome autosomal dominant, Job's syndrome, Job's syndrome (disorder), Jobs Syndrome, STAT3 deficiency, Syndrome, Hyper-IgE, Syndrome, Hyperimmunoglobulinemia E, Syndrome, Job's, Syndromes, Hyperimmunoglobulinemia E, autosomal dominant HIES, autosomal dominant hyper IgE syndrome, autosomal dominant hyperimmunoglobulin E syndrome, hyper Ig E syndrome, autosomal dominant, hyper-IgE recurrent infection syndrome, autosomal dominant, hyperimmunoglobulin E recurrent infection syndrome, autosomal dominant, hyperimmunoglobulin E syndrome, hyperimmunoglobulin E syndrome (disorder), hyperimmunoglobulin E syndrome type 1, immunodeficiency with defective leukocyte and lymphocyte function and with response to histamine-1 antagonist; Open Targets
Disease Ontology: DOID:3261; Disease Ontology
MeSH: C564135; MeSH
MeSH: C567925; MeSH
NCI Thesaurus: C126342; NCI Thesaurus
Orphanet: ORPHA:2314; Orphanet
Highest stage of its drugs: Phase 2, for 1 of its 3 drugs, from ClinicalTrials.gov; Open Targets

Drugs3

The drugs Open Targets' clinical reports study for the disease, the highest stage first, then the most trial reports. The stage is Open Targets', the highest any of its sources reports for the pair, and each row names the sources that report it.

Drug	ChEMBL ID	Type	Open Targets' stage	Stage reported by	Reports	Of them trials	Trials shown here	Source
RANITIDINE	CHEMBL1790041	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
BARICITINIB	CHEMBL2105759	Small molecule	Phase 1	ClinicalTrials.gov	1	1	1	Drugs and targets
OMALIZUMAB	CHEMBL1201589	Antibody	Phase 1	ClinicalTrials.gov	1	1	1	Drugs and targets

Targets4

The targets ChEMBL's mechanism records give for the disease's drugs, through Open Targets, the most drugs first. A mechanism record says what a drug acts on, not that the target causes the disease.

Target	Protein	Its drugs that act on it	Mechanisms	Highest stage of those drugs	Gene in Genes	Source
HRH2	Histamine H2 receptor	1	Histamine H2 receptor antagonist	Phase 2	https://conductscience.org/library/genes/3274	Drugs and targets
IGHE	Immunoglobulin heavy constant epsilon	1	Ig epsilon chain C region inhibitor	Phase 1	https://conductscience.org/library/genes/3497	Drugs and targets
JAK1	Tyrosine-protein kinase JAK1	1	Tyrosine-protein kinase JAK1 inhibitor	Phase 1	https://conductscience.org/library/genes/3716	Drugs and targets
JAK2	Tyrosine-protein kinase JAK2	1	Tyrosine-protein kinase JAK2 inhibitor	Phase 1	https://conductscience.org/library/genes/3717	Drugs and targets

Trials3

Trials an Open Targets clinical report links to the disease, posted results first, then the largest. A trial is shown when the release's checks confirm its link to the disease and Drugs and targets shows its link to one of the disease's drugs.

Trial	Title	Phase	Participants	Results first posted	Conditions	Drugs	Linked by	Source
NCT00527878	Effect of Ranitidine on Hyper-IgE Recurrent Infection (Job's) Syndrome	Phase 2	16	2013-02-04	JOB's Syndrome, Hyper-IgE Recurrent Infection Syndrome, Immune Deficiency	RANITIDINE	Its record names the disease	ClinicalTrials.gov
NCT07262983	Evaluating the Safety and Tolerability of Baricitinib in Patients With Job Syndrome With Lupus-Like Disease and/or Atopic Dermatitis	Phase 1	20		Hyper IgE Syndrome From STAT3 Mutation, Job s Syndrome, HIES, Lupus, Atopic Dermatitis	BARICITINIB	Its record names the disease	ClinicalTrials.gov
NCT00260702	Omalizumab to Treat Hyper-IgE (Job's) Syndrome	Phase 1	1		Hyper-IgE Syndrome, Job's Syndrome	OMALIZUMAB	Its record names the disease	ClinicalTrials.gov

Comparisons in its trials1

Comparisons Trial comparisons calculated in the trials shown here, most participants first. A comparison in one of these trials does not always compare a treatment for the disease, so read the two groups.

Comparison	Group 1, with the outcome	Group 2, with the outcome	Participants	Trial
Ranitidine vs Placebo: serious adverse events	4 of 12	4 of 11	23	NCT00527878

Source Trial comparisons

Papers from its trials0

The papers the trials shown here list on ClinicalTrials.gov as their results (RESULT) or as drawn from them (DERIVED), results first, newest first.

None in this release.

Data dates

The date of each source this release read.

Open Targets: 2026-09-18; Open Targets
Drug links and their checks, from Drugs and targets: 2026-09-18; Drugs and targets
ClinicalTrials.gov, from Trials: 2026-11-12; Trials
Comparisons, from Trial comparisons: 2026-03-23; Trial comparisons
PubMed, for the checks: 2026-11-06; PubMed
MeSH, for the checks: 2026-11-10; MeSH

Sources 6

ClinicalTrials.gov's posted results, through CTTI's AACTFreely available. Acknowledgement requested
ChEMBL, through Open TargetsCC BY-SA 3.0
ClinicalTrials.govNo license stated
NLM's MeSHNLM's terms
Open TargetsCC0 1.0
PubMedNLM's terms

Each source's terms

ClinicalTrials.gov's posted results, through CTTI's AACTClinicalTrials.gov data via Aggregate Analysis of ClinicalTrials.gov (AACT), CTTI. CTTI makes its materials freely available and requests acknowledgement. https://aact.ctti-clinicaltrials.org/documentation/299
ChEMBL, through Open TargetsCC BY-SA 3.0, for the parts from ChEMBL. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
ClinicalTrials.govnone stated
NLM's MeSHNLM's
Open TargetsCC0 1.0. Open Targets asks that users cite its latest paper: Buniello et al. 2025, Open Targets Platform: facilitating therapeutic hypotheses building in drug discovery, Nucleic Acids Research 53(D1):D1467, PMID 39657122. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
PubMedNLM's terms

ClinicalTrials.gov's data carry an international copyright outside the United States: https://clinicaltrials.gov/about-site/terms-conditions.

For software

Read this disease from software.

The record and each of its lists are JSON, twenty rows a page. No key.

GET https://conductscience.org/api/diseases/v1/MONDO_0007818

GET https://conductscience.org/api/diseases/v1/MONDO_0007818/drugs

API reference For AI assistants