The long and winding road to IgA deficiency: causes and consequences

D T Laura Vo Ngoc, Lizette Krist, Frans J van Overveld, Ger T Rijkers*

*Corresponding author for this work

Research output: Contribution to journalReview articleAcademicpeer-review

20 Citations (Scopus)


The most common humoral immunodeficiency is IgA deficiency. One of the first papers addressing the cellular and molecular mechanisms underlying IgA deficiency indicated that immature IgA-positive B-lymphocytes are present in these patients. This suggests that the genetic background for IgA is still intact and that class switching can take place. At this moment, it cannot be ruled out that genetic as well as environmental factors are involved. Areas covered: A clinical presentation, the biological functions of IgA, and the management of IgA deficiency are reviewed. In some IgA deficient patients, a relationship with a loss-of-function mutation in the TACI (transmembrane activator and calcium-modulating cyclophilin ligand interaction) gene has been found. Many other genes also have been associated. Gut microbiota are an important environmental trigger for IgA synthesis. Expert commentary: Expression of IgA deficiency is due to both genetic and environmental factors and a role for gut microbiota cannot be excluded.

Original languageEnglish
Pages (from-to)371-382
Number of pages12
JournalExpert Review of Clinical Immunology
Issue number4
Publication statusPublished - Apr 2017
Externally publishedYes

Bibliographical note

The University College Roosevelt covered all costs associated with the
development and the publishing of the present manuscript.


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