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Common Variable Immune Deficiency

Abstract

You are reading a NORD Rare Disease Report Abstract. NORD’s full collection of reports on over 1200 rare diseases is available to subscribers (click here for details). We are now also offering two full rare disease reports per day to visitors on our Web site.

NORD is very grateful to Charlotte Cunningham-Rundles, MD, PhD, Departments of Medicine, Pediatrics, The Immunology Institute, Mount Sinai School of Medicine, for assistance in the preparation of this report.

Synonyms of Common Variable Immune Deficiency

  • Acquired Hypogammaglobulinemia
  • common variable hypogammaglobulinemia
  • common variable immunodeficiency
  • CVI
  • CVID
  • immunodeficiency, common variable
  • late-onset immunoglobulin deficiency

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Common Variable Immune Deficiency (CVID) is a type of primary immunodeficiency, which is defined as an immune system dysfunction typically caused by a mutation in a gene or genes. The World Health Organization (WHO) recognizes more than 180 primary immunodeficiencies ranging from relatively common to quite rare.

CVID is one of the most prevalent of the symptomatic primary immunodeficiencies and manifests a wide variability of symptoms and range of severity. It is considered a diverse group of diseases of unknown cause (etiology) as many different immune system defects have been found to lead this syndrome. CVID is characterized by a low level of specific proteins (antibodies, also called immunoglobulins) in the fluid portion of the blood which results in a decreased ability to fight invading microorganisms, toxins, or other foreign substances. These immunoglobulins are produced by specialized white blood cells (B cells) as they mature into plasma cells.

The cause of CVID is unknown in at least 90% of cases, and a genetic cause has been identified in less than 10%. Sporadic cases, with no apparent history of the disorder in their family is the commonest form and may be caused by a complex interaction of environmental and genetic components (multifactorial inheritance), but genes that are involved in the development and function of B cells are believed to be the primary cause.

Common Variable Immune Deficiency Resources

(Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder [e.g., immune deficiency, autoimmune disorders affecting certain elements of the blood, etc.].)

NORD Member Organizations:

(To become a member of NORD, an organization must meet established criteria and be approved by the NORD Board of Directors.)

Other Organizations:

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

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