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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Common variable immunodeficiency


Other Names for this Disease
  • Common variable hypogamma-globulinemia
  • CVID
  • Hypogamma-globulinemia, acquired
  • Immunoglobulin deficiency, late-onset
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Overview



What is common variable immunodeficiency (CVID)?

What causes common variable immunodeficiency (CVID)?

How might common variable immunodeficiency be treated? 


What is common variable immunodeficiency (CVID)?

Common variable immunodeficiency is a group of disorders in which the immune system cannot make antibodies in response to foreign substances that may cause infection.  The main features that separate CVID from other immunodeficiencies are low levels of two specific antibodies, immunoglobulin A (IgA) and immunoglobulin G (IgG).  Individuals with this condition develop more frequent infections, particularly in the sinuses, lungs, and digestive tract. [1] [2]  CVID symptoms most commonly begin in early adulthood, but have been found in children as young as two years.  Though some cases of CVID are caused by known genetic factors, the cause of most cases is unknown. [3]  CVID can sometimes be treated by "replacing" antibodies into the body; this treatment is called "immunoglobulin therapy". [4]
Last updated: 10/25/2010

What causes common variable immunodeficiency (CVID)?

Common variable immunodeficiency is likely caused by a variety of factors. [2]  Approximately 10-20% of individuals with this condition have a known genetic cause. [3]  In most of these cases, common variable immunodeficiency is caused by mutations in the TAC1 gene.  This gene provides instructions that help B cells in the immune system to make antibodies. [5]  In a small percentage of cases, a mutation has been found in other genes that help B cells and T cells of the immune system to function properly, including the ICOS, CD19, or BAFFR genes.  In 80% of people with common variable immunodeficiency, the cause is unknown. [3]
Last updated: 10/25/2010

How might common variable immunodeficiency be treated? 

Common variable immunodeficiency is caused by a lack of antibodies, also known as immunoglobulins.  The treatment is to "replace" antibodies into the body.  This treatment is called "immunoglobulin therapy", and it can be given in a vein (intravenously, IVIG) or under the skin (subcutaneously, SCIG).  This treatment is not a cure; individuals need to regularly receive this therapy to support their immune system. [4]
Last updated: 10/25/2010

References
  1. Primary Immunodeficiency. National Institute of Child Health and Human Development (NICHD) . April 7, 2008; http://www.nichd.nih.gov/publications/pubs/primary_immuno.cfm#WhatisPrimaryImmunodeficiency. Accessed 10/12/2010.
  2. Schwartz RA, Modak RM, Modak P. Common Variable Immunodeficiency. eMedicine. March 16, 2010; http://emedicine.medscape.com/article/1051103-overview. Accessed 10/12/2010.
  3. Scharenberg, Andrew et al.. Common Variable Immune Deficiency Overview. GeneReviews. July 5, 2006; http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=gene&part=cvid. Accessed 10/12/2010.
  4. Frequently Asked Questions. Primary Immunodeficiency Resource Center. http://www.info4pi.org/aboutPI/index.cfm?Section=aboutPI&content=faq&CFID=35047926&CFTOKEN=43348784#499. Accessed 10/12/2010.
  5. Lee JJ, Ozcan E, Rauter I, Geha R. Transmembrane activator and calcium-modulator and cyclophilin ligand interactor mutations in common variable immunodeficiency. Current Opinion in Allergy and Clinical Immunology. 2008;