DEFICIÊNCIA SELETIVA DE IgA: ASPECTOS EPIDEMIOLÓGICOS, IMUNOPATOGÊNICOS, CLÍNICOS E TERAPÊUTICOS – UMA REVISÃO NARRATIVA
DEFICIÊNCIA SELETIVA DE IgA: ASPECTOS EPIDEMIOLÓGICOS, IMUNOPATOGÊNICOS, CLÍNICOS E TERAPÊUTICOS – UMA REVISÃO NARRATIVA
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DOI: https://doi.org/10.22533/at.ed.820821026050614
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Palavras-chave: Deficiência seletiva de IgA. Imunodeficiência primária. Doenças alérgicas.
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Keywords: Selective IgA deficiency. Primary immunodeficiency. Allergic diseases.
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Abstract: : Selective immunoglobulin A (IgA) deficiency is the most common primary immunodeficiency and presents wide clinical variability, ranging from asymptomatic individuals to patients with recurrent infections, allergies, autoimmunity, and transfusion reactions. This study conducted a narrative literature review on selective IgA deficiency, addressing epidemiology, immunopathogenesis, clinical manifestations, diagnosis, treatment, and prognosis. The search was performed in the PubMed/MEDLINE, SciELO, and Google Scholar databases in April 2026, using related descriptors in Portuguese, English, and Spanish. Findings demonstrate that the disorder results from failures in terminal B-cell differentiation and IgA class-switch recombination, influenced by genetic and immunoregulatory factors. The most common manifestations include respiratory and gastrointestinal infections, as well as a higher prevalence of autoimmune and allergic diseases, especially celiac disease and thyroid disorders. Diagnosis requires laboratory confirmation after four years of age and exclusion of secondary causes. Early recognition and longitudinal follow-up are essential to reduce morbidity and individualize clinical management.
- Benicio Vargas da Rosa
- Caroline Mrachma Suguiura
- Dayane Jaqueline Gross
- Guilherme Ribeiro
- Phelipe dos Santos Souza
- Rafaela Martins Narciso
- Sara Maysa Araújo Muller