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Selective immunoglobulin A deficiency: Wikis

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Selective immunoglobulin A deficiency
Classification and external resources

The dimeric IgA molecule. 1 H-chain, 2 L-chain, 3 J-chain, 4 secretory component
ICD-10 D80.2
ICD-9 279.01
OMIM 137100
DiseasesDB 29569
MedlinePlus 001476
eMedicine med/1159
MeSH D017098

Selective immunoglobulin A (IgA) deficiency is a relatively mild genetic immunodeficiency. People with this deficiency lack immunoglobulin A (IgA), a type of antibody that protects against infections of the mucous membranes lining the mouth, airways, and digestive tract. It is defined as an undetectable serum IgA level in the presence of normal serum levels of IgG and IgM. It is the most common of the primary antibody deficiencies.

Contents

Epidemiology

Prevalence varies by population, but is on the order of up to 1 in 333 people, [1] making it relatively common for a genetic disease.

It is more common in males than in females.[2]

Pathophysiology

There is an inherited inability to produce immunoglobulin A (IgA), a part of the body's defenses against infection at the body's surfaces (mainly the surfaces of the respiratory and digestive systems). As a result, bacteria at these locations are somewhat more able to cause disease.

Symptoms and diagnosis

People with selective IgA deficiency are usually asymptomatic,[3] but can have increased frequency of infections, particularly in the respiratory, digestive and genitourinary systems, for example, sinusitis and urinary tract infections. These infections are generally mild and would not usually lead to an in-depth workup except when unusually frequent. They may present with severe reactions including anaphylaxis to blood transfusions or intravenous immunoglobulin due to the presence of IgA in these blood products. When suspected, the diagnosis can be confirmed by laboratory measurement of IgA level in the blood. Patients have an increased susceptibility to pneumonia and recurrent episodes of other respiratory infections and a higher risk of developing autoimmune diseases in middle age.[4]

Although it has some similarities to common variable immunodeficiency, it does not present the same lymphocyte subpopulation abnormalities.[5]

Those patients with selective immunoglobulin A deficiency may be prone to recurrent infections when on hemodialysis.[6]

Treatment

The treatment consists of identification of comorbid conditions, preventive measures to reduce the risk of infection, and prompt and effective treatment of infections. Infections in an IgA-deficient person are treated as usual (i.e., with antibiotics). There is no treatment for the underlying disorder.

Prognosis

Prognosis is excellent, although there is an association with autoimmune disease. Of note, selective IgA deficiency can complicate the diagnosis of one such condition, celiac disease, as the deficiency masks the high levels of certain IgA antibodies usually seen in celiac disease.

As opposed to the related condition CVID, selective IgA deficiency is not associated with an increased risk of cancer.[7]

References

  1. ^ "IgA Deficiency: Immunodeficiency Disorders: Merck Manual Professional". http://www.merck.com/mmpe/sec13/ch164/ch164j.html. Retrieved 2008-03-01.  
  2. ^ Weber-Mzell D, Kotanko P, Hauer AC, et al. (March 2004). "Gender, age and seasonal effects on IgA deficiency: a study of 7293 Caucasians". Eur. J. Clin. Invest. 34 (3): 224–8. doi:10.1111/j.1365-2362.2004.01311.x. PMID 15025682. http://www3.interscience.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0014-2972&date=2004&volume=34&issue=3&spage=224.  
  3. ^ Tierney, Lawrence M.; McPhee, Stephen J.; Papadakis, Maxine A. (2008). Current medical diagnosis & treatment, 2008. McGraw-Hill Medical. pp. 694. ISBN 0-07-149430-8.  
  4. ^ Koskinen S (1996). "Long-term follow-up of health in blood donors with primary selective IgA deficiency.". J Clin Immunol 16 (3): 165–70. doi:10.1007/BF01540915. PMID 8734360.  
  5. ^ Litzman J, Vlková M, Pikulová Z, Stikarovská D, Lokaj J (February 2007). "T and B lymphocyte subpopulations and activation/differentiation markers in patients with selective IgA deficiency". Clin. Exp. Immunol. 147 (2): 249–54. doi:10.1111/j.1365-2249.2006.03274.x. PMID 17223965. PMC 1810464. http://www3.interscience.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0009-9104&date=2007&volume=147&issue=2&spage=249.  
  6. ^ Kuo MC, Hwang SJ, Chang JM, Tsai JC, Tsai JH, Lai YH (December 1998). "Recurrent infections in haemodialysis patients--do not forget selective immunoglobulin A deficiency". Nephrol. Dial. Transplant. 13 (12): 3220–2. PMID 9870497. http://ndt.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=9870497.  
  7. ^ 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. PMID 12452841. http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1906562.  

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