Journal of Clinical Immunology

, Volume 33, Issue 4, pp 742–747 | Cite as

Clinical Symptoms in Adults with Selective IgA Deficiency: A Case-Control Study

  • G. H. Jorgensen
  • A. Gardulf
  • M. I. Sigurdsson
  • S. Th. Sigurdardottir
  • I. Thorsteinsdottir
  • S. Gudmundsson
  • L. Hammarström
  • B. R. Ludviksson
Original Research


Selective IgA deficiency (SIgAD) is the most common primary immunodeficiency in Caucasians with a prevalence of 1/600 and is generally considered a mild disorder. In this study, the clinical status of 32 adults with SIgAD was investigated and compared to 63 age- and gender matched controls, randomly selected from a population database. The SIgAD individuals reported significantly more often contracting various upper and lower respiratory infections, with 8 (25.0 %) having been diagnosed with ≥1 pneumonia in the preceding two years, compared to one (1.6 %) control (p < 0.001). Furthermore, the SIgAD individuals were found to have increased proneness to infections and increased prevalence of allergic diseases and autoimmunity, with a total of 84.4 % being affected by any of these diseases, compared to 47.6 % of the controls (p < 0.01). This study challenges the common statement of SIgAD being a mild form of immunodeficiency. It also highlights the importance of using matched controls in PID clinical research to better detect clinically important manifestations.


Primary immunodeficiency IgA deficiency infections autoimmunity allergy 



We are grateful to all the individuals participating in the study, and to Hilary Hocking, Östersund, Sweden, for language revision.

This study was supported by the Landspitali University Hospital Research Fund, the Icelandic Research Fund, Karolinska Institutet (KI Fonder), Stockholm, Sweden and Division of Research and Development, Primary Care, County Council of Uppsala, Sweden.

Conflict of Interest

No conflict of interest is reported for any of the authors.


  1. 1.
    ESID. Diagnostic criteria for PID. European Society for Immunodeficiencies [Internet]. Available at:
  2. 2.
    Yel L. Selective IgA deficiency. J Clin Immunol. 2010;30(1):10–6.PubMedCrossRefGoogle Scholar
  3. 3.
    Aghamohammadi A, Cheraghi T, Gharagozlou M, Movahedi M, Rezaei N, Yeganeh M, et al. IgA deficiency: correlation between clinical and immunological phenotypes. J Clin Immunol. 2009;29(1):130–6.PubMedCrossRefGoogle Scholar
  4. 4.
    Koskinen S. Long-term follow-up of health in blood donors with primary selective IgA deficiency. J Clin Immunol. 1996;16(3):165–70.PubMedCrossRefGoogle Scholar
  5. 5.
    Hammarström L, Edvard Smith CI. Genetic approach to common variable immunodeficiency and IgA deficiency. In: Ochs HD, Edvard Smith CI, Puck JM, editors. Primary immunodeficiency diseases: a molecular and genetic approach. 2nd ed. New York: Oxford University Press; 2007. p. 313–25.Google Scholar
  6. 6.
    Ulfarsson J, Gudmundsson S, Birgisdottir B. Selective serum IgA deficiency in Icelanders. Frequency, family studies and Ig levels. Acta Med Skand. 1982;211(6):481–7.CrossRefGoogle Scholar
  7. 7.
    Jorgensen GH, Thorsteinsdottir I, Gudmundsson S, Hammarstrom L, Ludviksson BR. Familial aggregation of IgAD and autoimmunity. Clin Immunol. 2009;131(2):233–9.PubMedCrossRefGoogle Scholar
  8. 8.
    Jorgensen GH, Arnlaugsson S, Theodors A, Ludviksson BR. Immunoglobulin a deficiency and oral health status: a case-control study. J Clin Periodontol. 2010;37(1):1–8.PubMedCrossRefGoogle Scholar
  9. 9.
    Edwards E, Razvi S, Cunningham-Rundles C. IgA deficiency: clinical correlates and responses to pneumococcal vaccine. Clin Immunol. 2004;111(1):93–7.PubMedCrossRefGoogle Scholar
  10. 10.
    Shkalim V, Monselize Y, Segal N, Zan-Bar I, Hoffer V, Garty BZ. Selective IgA deficiency in children in Israel. J Clin Immunol. 2010;30(5):761–5.PubMedCrossRefGoogle Scholar
  11. 11.
    Mestecky J, Hammarström L. IgA-associated diseases. In: Kaetzel CS, editor. Mucosal immune defense: immunoglobulin A. New York: Springer; 2007. p. 321–44.CrossRefGoogle Scholar
  12. 12.
    Agarwal S, Mayer L. Pathogenesis and treatment of gastrointestinal disease in antibody deficiency syndromes. J Allergy Clin Immunol. 2009;124(4):658–64.PubMedCrossRefGoogle Scholar
  13. 13.
    Jacob CMA, Pastorino AC, Fahl K, Carneiro-Sampaio M, Monteiro RC. Autoimmunity in IgA deficiency: revisiting the role of IgA as a silent housekeeper. J Clin Immunol. 2008;28 Suppl 1:S56–61.PubMedCrossRefGoogle Scholar
  14. 14.
    Janzi M, Kull I, Sjöberg R, Wan J, Melén E, Bayat N, et al. Selective IgA deficiency in early life: association to infections and allergic diseases during childhood. Clin Immunol. 2009;133(1):78–85.PubMedCrossRefGoogle Scholar
  15. 15.
    Arason GJ, Jorgensen GH, Ludviksson BR. Primary immunodeficiency and autoimmunity: lessons from human diseases. Scand J Immunol. 2010;71(5):317–28.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • G. H. Jorgensen
    • 1
    • 2
  • A. Gardulf
    • 3
  • M. I. Sigurdsson
    • 1
  • S. Th. Sigurdardottir
    • 2
  • I. Thorsteinsdottir
    • 4
  • S. Gudmundsson
    • 5
  • L. Hammarström
    • 3
  • B. R. Ludviksson
    • 1
    • 2
  1. 1.Department of MedicineUniversity of IcelandReykjavíkIceland
  2. 2.Department of ImmunologyLandspitali University HospitalReykjavíkIceland
  3. 3.Division of Clinical Immunology at the Department of Laboratory MedicineKarolinska Institutet at Karolinska University Hospital HuddingeStockholmSweden
  4. 4.Department of BiochemistryLandspitali University HospitalReykjavíkIceland
  5. 5.Icelandic Blood BankReykjavíkIceland

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