Common variable immunodeficiency

Common variable immunodeficiency
Common variable immunodeficiency
Classification and external resources
ICD-10 D83
ICD-9 279.06
OMIM 240500
DiseasesDB 3274
eMedicine ped/444 derm/870
MeSH D017074

Common variable immunodeficiency (CVID) (also known as Acquired hypogammaglobulinemia[1]) is a group of approximately 150 primary immunodeficiencies (PIDs), which have a common set of symptoms (including hypogammaglobulinemia)[2] but which have different underlying causes.

Common variable immunodeficiency is the most commonly encountered primary immunodeficiency.[3]


Causes and types

CVID is believed to be a genetically determined primary immune defect; however, the underlying causes are different. The result of these defects is that the patient doesn't produce sufficient antibodies in response to exposure to pathogens. As a result, the patient's immune system fails to protect them against common bacterial and viral (and occasionally parasitic and protozoan) infections. The net result is that the patient is susceptible to illness.

In CVID, the B cells are affected. In severe combined immunodeficiency (SCID), a more severe condition than CVID, diagnosed in infancy, both parts of the immune system (the cellular and humoral system) are affected, hence its classified as combined immunodeficiency.

CVID appears to include a number of defects, some of which have been identified. For the majority, the genetic causes are still unknown.

ICOS, TACI and CD19 have been identified as candidates.[4][5]

It is possible that environmental agents or a virus provoke the immune defect, due to genetic predisposition, but this has not been clarified.

Types include:

Type OMIM Gene
CVID1 607594 ICOS
CVID2 240500 TACI
CVID3 613943 CD19
CVID4 613494 TNFRSF13C
CVID5 613495 CD20
CVID6 613496 CD81

Clinical Features

Signs and Symptoms of CVID include:


Diagnosis of CVID is usually made by demonstrating low levels of immunoglobulins in the serum. Diagnosis may be made rapidly, but is often delayed; it is usually made in the second or third decade of life after referral to an immunologist.

Diagnosis of CVID is a diagnosis of exclusion.[7]

It presents similar to X-linked agammaglobulinemia, but the conditions can be distinguished with flow cytometry.[8]

Associated conditions

As with several other immune cell disorders, CVID may predispose to lymphoma or possibly stomach cancer.[9] There also appears to be a predilection for autoimmune diseases, with a risk of up to 25%. Autoimmune destruction of platelets or red blood cells are the most common of these.


Treatment usually consists of immunoglobulin therapy, which is an injection of human antibodies harvested from plasma donations:

  • intravenous immunoglobulin (IVIG, most common treatment in the US)[10]
  • subcutaneous immunoglobulin G (SCIG, relatively new treatment in the US and UK)
  • intramuscular immunglobulin (IMIG, less effective, painful)

This is not a cure, but it strengthens immunity by ensuring that the patient has "normal" levels of antibodies, which helps to prevent recurrent upper respiratory infections.

IG therapy can't be used if the patient has anti-IgA antibodies but in this case, products low in IgA can be used; subcutaneous delivery also is a means of permitting such patients to have adequate antibody replacement.

IVIG treatment can be received by patients with a complete IgA deficiency if the IgA is completely removed from the treatment.


Some CVID patients may experience reactions to IG therapies; reactions may include:

  • anaphylactic shock (very rare)
  • hives (rare)
  • chills
  • difficulty breathing
  • headache (relatively common, may be relieved by an antihistamine, paracetamol/acetaminophen, or an anti-inflammatory (naproxen, advil, aspirin)
  • nausea (common in IVIG)
  • fever (common in IVIG and rare in SCIG)
  • aseptic meningitis (rare)
  • severe fatigue (common in IVIG)
  • muscle aches and pain, or joint pain
  • thrombotic events (rare)
  • swelling at the insertion site (common in SCIG)

Patients should not receive therapy if they are fighting an active infection as this increases the risk of reaction. Also, patients changing from one brand of product to another may be at higher risk of reaction for the first couple of treatments on the new brand.

Reactions can be minimized by taking an antihistamine and/or hydrocortisone and some paracetamol/acetaminophen/anti-inflammatory (naproxen, advil, aspirin) prior to treatment; patients should also be thoroughly hydrated and continue to drink water before, after and during treatment (if possible). IVIG should be prepared soon before IVIG infusion. Patient using a heating pad or warm blanket can help alleviate chills.


Research is currently focussing on genetic analysis, and in differentiating between the various different disorders in order to allow a cure to be developed. Cures are likely to be genetic in nature, repairing faulty genes and allowing the individual to start producing antibodies. Funding for research in the US is provided by the National Institutes of Health. Key research in the UK is funded by the Primary Immunodeficiency Association (PiA), and funding is raised through the annual Jeans for Genes campaign.


CVID has an estimated prevalence of about 1:50,000.[11] The typical patient is between 20 and 40, and males and females are equally affected. About 20% of patients are diagnosed in childhood.


Charles Janeway, Sr. is generally credited with the first description of a case of CVID in 1953.[12]


  1. ^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0. :84
  2. ^ "common variable immunodeficiency" at Dorland's Medical Dictionary
  3. ^ Park MA, Li JT, Hagan JB, Maddox DE, Abraham RS (August 2008). "Common variable immunodeficiency: a new look at an old disease". Lancet 372 (9637): 489–502. doi:10.1016/S0140-6736(08)61199-X. PMID 18692715. 
  4. ^ Salzer U, Neumann C, Thiel J, et al. (2008). "Screening of functional and positional candidate genes in families with common variable immunodeficiency". BMC Immunol. 9: 3. doi:10.1186/1471-2172-9-3. PMC 2268914. PMID 18254984. 
  5. ^ Blanco-Quirós A, Solís-Sánchez P, Garrote-Adrados JA, Arranz-Sanz E (2006). "Common variable immunodeficiency. Old questions are getting clearer". Allergol Immunopathol (Madr) 34 (6): 263–75. doi:10.1157/13095875. PMID 17173844. 
  6. ^ Sanger, David E. [ "An Investigation of Coping and Psychosocial Functioning in Persons with Common Variable Immunodeficiency (CVID)"], Barts and The London NHS Trust, 2003, accessed August 7, 2011.
  7. ^ Common Variable Immunodeficiency : Article by C Lucy Park at eMedicine
  8. ^ Common Variable Immunodeficiency at Merck Manual of Diagnosis and Therapy Professional Edition
  9. ^ Mellemkjaer L, Hammarstrom L, Andersen V, et al. (2002). "Cancer risk among patients with IgA deficiency or common variable immunodeficiency and their relatives: a combined Danish and Swedish study". Clin. Exp. Immunol. 130 (3): 495–500. doi:10.1046/j.1365-2249.2002.02004.x. PMC 1906562. PMID 12452841. 
  10. ^ Pourpak Z, Aghamohammadi A, Sedighipour L, et al. (2006). "Effect of regular intravenous immunoglobulin therapy on prevention of pneumonia in patients with common variable immunodeficiency" (abstract). J Microbiol Immunol Infect 39 (2): 114–20. PMID 16604243. 
  11. ^ Common Variable Immunodeficiency : Article by Robert A Schwartz at eMedicine
  12. ^ Janeway CA, Apt L, Gitlin D. Agammaglobulinemia. Trans Assoc Am Physicians 1953;66:200-2. PMID 13136263

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