SELECTIVE IgA DEFICIENCY IN CHILDREN
DOI:
https://doi.org/10.46793/PP260122006PKeywords:
primary immunodeficiency, infections, IgA deficiencyAbstract
Selective IgA deficiency is the most common primary immunodeficiency, and is defined as a serum IgA level lower than 0.07 g/L with a lack of secretory IgA, in girls or boys under 4 years of age. These children have normal IgG and IgM levels with an adequate antibody response to vaccination. IgA deficiency in children increases susceptibility to respiratory and gastrointestinal infections, which may be prolonged or recurrent. Many studies indicate a clear association between allergic diseases and IgA deficiency. Common autoimmune diseases associated with selective IgA deficiency are: idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia, type 1 diabetes mellitus, vitiligo, Graves' disease, thyroiditis, rheumatoid arthritis, systemic lupus erythematosus, and celiac disease. The occurrence of malignancy in these patients is sporadic, and the most common are gastric adenocarcinoma and B-cell lymphoma. Also, selective IgA deficiency can be associated with other primary immunodeficiencies, such as IgG2 and IgG3 subclass deficiencies. There are no clear recommendations for specific treatment of patients with selective IgA deficiency. These patients require clinical and laboratory monitoring for prolonged and/or recurrent infections, treatment of associated allergic and autoimmune manifestations, need for use of a polyvalent pneumococcal vaccine, and substitution by intravenous or subcutaneous immunoglobulins.
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