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Common variable immunodeficiency

Orpha number ORPHA1572
Prevalence of rare diseases 1-9 / 100 000
Inheritance
  • Autosomal dominant
  • Autosomal recessive
Age of onset Adolescence / Young adulthood
ICD 10 code
  • D83
MIM number
Synonym(s) CVID

Summary

Common variable immunodeficiency (CVID) is marked by hypogammaglobulinemia of all isotypes that fluctuates from one patient to another and overtime. Onset usually occurs during the second or third decade, with recurring respiratory and sometimes intestinal bacterial infections and granulomatous, and autoimmune manifestations. Prevalence ranges from 1:10,000 to 1:20,000. The genetic origin of the syndrome is complex. A few patients (<2%) suffer from a deficiency in inducible co-stimulator (ICOS) protein. Patients are mainly treated with regular injections of polyvalent immunoglobulin. *Author: Pr. A. Fischer (January 2005)*.

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