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Immunoglobulin A Deficiency

Immunoglobulin A Deficiency is a topic covered in the Select 5-Minute Pediatrics Topics.

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Serum IgA <7 mg/dL and a normal serum IgG and IgM in patients >4 years of age

Risk Factors


  • Exact pattern of inheritance remains unclear; however, the following associations may occur:
    • 22q11 deletion syndrome
    • 18q syndrome
    • Partial deletions in the long or short arm and ring forms of chromosome 18
    • Also associated with HLA-A1, HLA-A2, B8, DR3, DQ2 (8.1), and Dw3
    • Also associated with non–MHC-associated genes involved in autoimmunity including IFIH1 on chromosome 2q24, CLEC16A on chromosome 16


  • Failure of B lymphocyte differentiation into plasma cells producing IgA
  • Block may be due to
    • Defect in T-helper cells
    • Antigen-presenting cells
    • B cells
    • Lack of effects from various cytokines, including IL-21, IL-4, IL-6, IL-7, or IL-10

Commonly Associated Conditions

Increased association with the following:

  • Atopy
  • Sinopulmonary infections
  • GI infections (especially Giardia lamblia)
  • Inflammatory bowel disease (Crohn disease and ulcerative colitis)
  • Celiac disease
  • Nodular lymphoid hyperplasia
  • Malignancy
  • Autoimmune illnesses
    • Systemic lupus erythematosus
    • Immune endocrinopathies (e.g., Graves disease, type 1 diabetes)
    • Autoimmune hematologic conditions
    • Chronic active hepatitis

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Cabana, Michael D., editor. "Immunoglobulin a Deficiency." Select 5-Minute Pediatrics Topics, 7th ed., Wolters Kluwer Health, 2015. 5minute, www.unboundmedicine.com/5minute/view/Select-5-Minute-Pediatric-Consult/14162/all/Immunoglobulin_A_Deficiency.
Immunoglobulin A Deficiency. In: Cabana MD, ed. Select 5-Minute Pediatrics Topics. 7th ed. Wolters Kluwer Health; 2015. https://www.unboundmedicine.com/5minute/view/Select-5-Minute-Pediatric-Consult/14162/all/Immunoglobulin_A_Deficiency. Accessed June 16, 2019.
Immunoglobulin A Deficiency. (2015). In Cabana, M. D. (Ed.), Select 5-Minute Pediatrics Topics. Available from https://www.unboundmedicine.com/5minute/view/Select-5-Minute-Pediatric-Consult/14162/all/Immunoglobulin_A_Deficiency
Immunoglobulin a Deficiency [Internet]. In: Cabana MD, editors. Select 5-Minute Pediatrics Topics. Wolters Kluwer Health; 2015. [cited 2019 June 16]. Available from: https://www.unboundmedicine.com/5minute/view/Select-5-Minute-Pediatric-Consult/14162/all/Immunoglobulin_A_Deficiency.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Immunoglobulin A Deficiency ID - 14162 ED - Cabana,Michael D, BT - Select 5-Minute Pediatrics Topics UR - https://www.unboundmedicine.com/5minute/view/Select-5-Minute-Pediatric-Consult/14162/all/Immunoglobulin_A_Deficiency PB - Wolters Kluwer Health ET - 7 DB - 5minute DP - Unbound Medicine ER -