oa Current Allergy & Clinical Immunology - IgA deficiency : facts and fallacies : primary immunodeficiency disorders

Volume 28, Issue 2
  • ISSN : 1609-3607



Selective IgA deficiency (IgAD) is the most common primary genetic immune defect, with high prevalence in Western countries and relatively low prevalence in the East. The laboratory definition of IgAD is based on the measured serum component. However, the important manifestations of deficiency of IgA, the most prevalent of human antibodies, relate to the absence of secretory IgA, which covers a vast surface area of the human body. Diagnostic criteria by the International Union of Immunological Societies (IUIS) define IgAD as very low or absent levels of IgA (< 0.07 g/L) in a child older than 4 years of age. IgAD is asymptomatic in the majority but if symptoms do occur, these generally manifest with mild infections of the respiratory tract with encapsulated bacteria or intermittent diarrhoea with . Symptomatic patients should be investigated for deficient polysaccharide antibody responses, IgG subclass deficiency or allergies because of increased frequency of these conditions co-existing in these patients. IgAD also increases risk for autoimmunity and malignancies but surveillance guidelines are currently lacking. Treatment of IgAD includes reassurance of the asymptomatic patient, optimal control of allergies, prophylactic antibiotics and very rarely immunoglobulin replacement.

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