Orphanet: Common variable immunodeficiency

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Common variable immunodeficiency

Disease definition

Common variable immunodeficiency (CVID) comprises a heterogeneous group of diseases characterized by a significant hypogammaglobulinemia of unknown cause, failure to produce specific antibodies after immunizations and susceptibility to bacterial infections, predominantly caused by encapsulated bacteria.


Classification level: Disorder
  • Synonym(s):
    • CVID
    • Idiopathic immunoglobulin deficiency
    • Primary antibody deficiency
    • Primary hypogammaglobulinemia
  • Prevalence: 1-9 / 100 000
  • Inheritance: Autosomal dominant or Autosomal recessive or Not applicable 
  • Age of onset: All ages
  • ICD-10: D83.0  D83.1  D83.2  D83.8  D83.9
  • OMIM: 146830  240500  607594  613493  613494  613495  613496  614699  615577  616576
  • UMLS: C0009447
  • MeSH: D017074
  • GARD: 6140
  • MedDRA: 10021449
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