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Immunodeficiency due to interleukin-1 receptor-associated kinase-4 deficiency
1 OMIM reference -
1 associated gene
10 connected diseases
3 signs/symptoms
Disease Type of connection
Pediatric systemic lupus erythematosus
Pyogenic bacterial infections due to MyD88 deficiency
Waldenström macroglobulinemia
Autosomal dominant hypohidrotic ectodermal dysplasia
Common variable immunodeficiency
Anhidrotic ectodermal dysplasia - immunodeficiency - osteopetrosis - lymphedema
Behçet disease
Hypohidrotic ectodermal dysplasia with immunodeficiency
Incontinentia pigmenti
X-linked mendelian susceptibility to mycobacterial diseases due to IKBKG deficiency
- IRAK4 deficiency

Classification (Orphanet):
- Rare genetic disease
- Rare immune disease

Classification (ICD10):
- Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism -

Epidemiological data:
Class of prevalence: <1 / 1 000 000
Average age onset: childhood
Average age of death: any age
Type of inheritance: autosomal recessive
External references:
1 OMIM reference -
No MeSH references

Gene symbol UniProt reference OMIM reference
IRAK4 Q9NWZ3606883
Very frequent
- Anomalies of the lymphatic system
- Immunodeficiency / increased susceptibility to infections / recurrent infections
- Polynuclear cells / neutrophils anomalies / neutropenia