What is the global prevalence of immunoglobulin A deficiency (IgAD)?

Updated: May 15, 2018
  • Author: Marina Y Dolina, MD; Chief Editor: Michael A Kaliner, MD  more...
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Answer

Factors associated with the prevalence of IgAD include a family history of IgAD and the country of origin. Family studies using IgAD blood donors as probands show that first-degree relatives have a 7.5% prevalence rate of IgAD, which is 38-fold higher than that of unrelated donors. [37] The serological prevalence of IgAD varies 100-fold among populations. Prevalences, in decreasing order, are as follows:

  • Arabian peninsula - One in 142 persons

  • Spain - One in 170 persons

  • Eastern Nigeria - One in 255 persons

  • Finland - One in 396 persons

  • Czech Republic - One in 408 persons

  • Basque regions of Spain and France - One in 521 persons

  • Canada - One in 531 persons [16]

  • Iceland - One in 533 persons

  • England - One in 875 persons

  • Brazil - One in 965 persons

  • France - One in 3040 persons

  • China (Han) - One in 2600 persons

  • China (Zhuang) - One in 5300 persons

  • Japan - One in 14,850-18,500 persons

  • Sweden - Approximately 20,000 persons affected

  • United Kingdom - Approximately 120,000 persons affected [36]

  • India - None of 3818 blood donors screened; 257 (6.7%) had partial IgAD [38]

Isolated IgAD is present in a minority of cases of transient hypogammaglobulinemia of infancy. Of a series of 40 patients presenting with recurrent responsive infections, otitis media, bronchitis or bronchial asthma, or recurrent gastroenteritis when aged 4-29 months, only 1 had isolated IgAD, 10 had reduced IgG and IgA levels, and 6 had diminished IgA and IgM levels. [39] The majority recovered immunoglobulin levels by age 3 years, but 3 had persistently low IgG and IgA levels.

A study performed by Weber-Mzell et al on 7293 healthy white volunteers demonstrated an IgAD prevalence of 0.21% (definition of IgAD was level < 0.07g/L). [40] The same study showed seasonal fluctuations of serum IgA (SIgA) concentration; levels of SIgA increased in winter.

The Latin American Group for Primary Immunodeficiency Diseases is using a registry approach to increase disorder recognition in their region. Among a total of 3321 patients registered, 53% had an antibody deficiency, of which IgAD was the more frequent phenotype. [41]


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