Skip to main content

Advertisement

SpringerLink
Log in

IgA Deficiency: Correlation Between Clinical and Immunological Phenotypes

  • Published:
Journal of Clinical Immunology Aims and scope Submit manuscript

Abstract

Background

IgA deficiency (IGAD) is the most common primary antibody deficiency. Although many affected individuals have no apparent symptom, selected patients suffer from recurrent mucosal infections, allergies, and autoimmune diseases. We aimed to investigate the clinical features in relation to immune function of Iranian patients with symptomatic IGAD.

Methods

Thirty-seven patients (21 male and 16 female), aged 4–32 years, were evaluated in this study. Patients were followed for a total of 131 patient years with a mean follow-up of 3.5 years per patient.

Results

The most prevalent presentations were recurrent infections occurring in 27 subjects, followed by allergy in eight cases and autoimmunity in two patients. However, during the follow-up period, 35 patients developed infections in respiratory and gastrointestinal tracts, necessitating medical care. Apart from infections, allergy was the most frequent complaint (31 cases); the major features were asthma, atopic dermatitis, and allergic rhinoconjunctivitis. Autoimmune diseases were documented in ten cases; thyroiditis was the most common. In 31 patients who received unconjugated pneumococcal polyvalent vaccine, antibody response against polysaccharide antigen was measured before and 28 days after vaccination. One fourth of vaccinated patients were hyporesponsive to vaccine; four of these patients developed bronchiectasis. The patients with IGAD were classified into two groups: group 1 (14 cases) consisted of patients with IGAD and other associated immune defects, such as immunoglobulin G (IgG) subclass deficiency and defective specific antibody production. Group 2 (23 cases) had isolated IGAD without other immunological abnormalities. There was a significantly increased number of lower respiratory tract infections in group 1 compared with group 2 (P = 0.006). Moreover, four patients of group 1 had bronchiectasis whereas none of the patients in group 2 developed this complication (P = 0.015).

Conclusion

Subclassification of IGAD regarding the existence of associated immune defects is useful in terms of morbidity and planning for medical care. IgA-deficient patients with concomitant immune defects such as defects in specific antibody production have higher rates of recurrent infections and bronchiectasis, which necessitates more effective monitoring.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Hammarstrom L, Vorechovsky I, Webster D. Selective IgA deficiency (SIgAD) and common variable immunodeficiency (CVID). Clin Exp Immunol. 2000;120(2):225–31. doi:10.1046/j.1365-2249.2000.01131.x.

    Article  PubMed  CAS  Google Scholar 

  2. Bonilla FA, Bernstein IL, Khan DA, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. Ann Allergy Asthma Immunol. 2005;94(5):S1–63.

    Article  PubMed  Google Scholar 

  3. Conley ME, Notarangelo LD, Etzioni A. Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies). Clin Immunol. 1999;93(3):190–7. doi:10.1006/clim.1999.4799.

    Article  PubMed  CAS  Google Scholar 

  4. al-Attas RA, Rahi AH. Primary antibody deficiency in Arabs: first report from eastern Saudi Arabia. J Clin Immunol. 1998;18(5):368–71. doi:10.1023/A:1023247117133.

    Article  PubMed  CAS  Google Scholar 

  5. Cunningham-Rundles C. Physiology of IgA and IgA deficiency. J Clin Immunol. 2001;21(5):303–9. doi:10.1023/A:1012241117984.

    Article  PubMed  CAS  Google Scholar 

  6. Kanoh T, Mizumoto T, Yasuda N, et al. Selective IgA deficiency in Japanese blood donors: frequency and statistical analysis. Vox Sang. 1986;50(2):81–6.

    PubMed  CAS  Google Scholar 

  7. Schaffer FM, Monteiro RC, Volanakis JE, Cooper MD. IgA deficiency. Immunodefic Rev. 1991;3(1):15–44.

    PubMed  CAS  Google Scholar 

  8. Burrows PD, Cooper MD. IgA deficiency. Adv Immunol. 1997;65:245–76. doi:10.1016/S0065-2776(08)60744-0.

    Article  PubMed  CAS  Google Scholar 

  9. Hanson LA, Bjorkander J, Carlsson B, Roberton D, Soderstrom T. The heterogeneity of IgA deficiency. J Clin Immunol. 1988;8(3):159–62. doi:10.1007/BF00917561.

    Article  PubMed  CAS  Google Scholar 

  10. Burgio GR, Duse M, Monafo V, Ascione A, Nespoli L. Selective IgA deficiency: clinical and immunological evaluation of 50 pediatric patients. Eur J Pediatr. 1980;133(2):101–6. doi:10.1007/BF00441577.

    Article  PubMed  CAS  Google Scholar 

  11. Crabbe PA, Heremans JF. The significance of local IgA in the physiology of the intestinal mucosa. Folia Med Neerl. 1969;12(3):100–6.

    PubMed  CAS  Google Scholar 

  12. Heneghan MA, Stevens FM, Cryan EM, Warner RH, McCarthy CF. Celiac sprue and immunodeficiency states: a 25-year review. J Clin Gastroenterol. 1997;25(2):421–5. doi:10.1097/00004836-199709000-00004.

    Article  PubMed  CAS  Google Scholar 

  13. Sloper KS, Brook CG, Kingston D, Pearson JR, Shiner M. Eczema and atopy in early childhood: low IgA plasma cell counts in the jejunal mucosa. Arch Dis Child. 1981;56(12):939–42.

    Article  PubMed  CAS  Google Scholar 

  14. Edwards E, Razvi S, Cunningham-Rundles C. IgA deficiency: clinical correlates and responses to pneumococcal vaccine. Clin Immunol. 2004;111(1):93–7. doi:10.1016/j.clim.2003.12.005.

    Article  PubMed  CAS  Google Scholar 

  15. Strober W, Sneller MC. IgA deficiency. Ann Allergy. 1991;66(5):363–75.

    PubMed  CAS  Google Scholar 

  16. Wernette CM, Frasch CE, Madore D, et al. Enzyme-linked immunosorbent assay for quantitation of human antibodies to pneumococcal polysaccharides. Clin Diagn Lab Immunol. 2003;10(4):514–9. doi:10.1128/CDLI.10.4.514-519.2003.

    Article  PubMed  CAS  Google Scholar 

  17. Vendrell M, de Gracia J, Rodrigo MJ, et al. Antibody production deficiency with normal IgG levels in bronchiectasis of unknown etiology. Chest 2005;127(1):197–204. doi:10.1378/chest.127.1.197.

    Article  PubMed  CAS  Google Scholar 

  18. Standardization of spirometry-1987 update. Statement of the American Thoracic Society. Am Rev Respir Dis. 1987;136(5):1285–98.

    Google Scholar 

  19. Aghamohammadi A, Mohammadi J, Parvaneh N, Rezaei N, Moin M, Espanol T, et al. Progression of selective IgA deficiency to common variable immunodeficiency. Int Arch Allergy Immunol 2008;147(2):87–92. doi:10.1159/000135694.

    Article  PubMed  CAS  Google Scholar 

  20. Schroeder HW Jr, Zhu ZB, March RE, et al. Susceptibility locus for IgA deficiency and common variable immunodeficiency in the HLA-DR3, -B8, -A1 haplotypes. Mol Med. 1998;4(2):72–86.

    PubMed  CAS  Google Scholar 

  21. Castigli E, Wilson SA, Garibyan L, et al. TACI is mutant in common variable immunodeficiency and IgA deficiency. Nat Genet 2005;37(8):829–34. doi:10.1038/ng1601.

    Article  PubMed  CAS  Google Scholar 

  22. Salzer U, Chapel HM, Webster AD, et al. Mutations in TNFRSF13B encoding TACI are associated with common variable immunodeficiency in humans. Nat Genet. 2005;37(8):820–8. doi:10.1038/ng1600.

    Article  PubMed  CAS  Google Scholar 

  23. Hammarström LSC. Genetic approach to common variable immunodeficiency and IgA deficiency. In: Ochs HDSC, Puck JM, editors. Primary immunodeficiency diseases: a molecular and genetic approach. 2nd ed. New York: Oxford University Press; 2007. p. 313–25.

    Google Scholar 

  24. Rezaei N, Aghamohammadi A, Moin M, Pourpak Z, Movahedi M, Gharagozlou M, et al. Frequency and clinical manifestations of patients with primary immunodeficiency disorders in Iran: update from the Iranian Primary Immunodeficiency Registry. J Clin Immunol. 2006;26(6):519–32. doi:10.1007/s10875-006-9047-x.

    Article  PubMed  Google Scholar 

  25. Saghafi S, Pourpak Z, Aghamohammadi A, Pourfathollah AA, Samadian A, Farghadan M, et al. Selective immunoglobulin a deficiency in Iranian blood donors; prevalence, laboratory and clinical findings. Iran J Allergy Asthma Immunol. 2008;7(3):157–62.

    PubMed  Google Scholar 

  26. Ozkan H, Atlihan F, Genel F, Targan S, Gunvar T. IgA and/or IgG subclass deficiency in children with recurrent respiratory infections and its relationship with chronic pulmonary damage. J Investig Allergol Clin Immunol. 2005;15(1):69–74.

    PubMed  CAS  Google Scholar 

  27. Stead A, Douglas JG, Broadfoot CJ, Kaminski ER, Herriot R. Humoral immunity and bronchiectasis. Clin Exp Immunol. 2002;130(2):325–30. doi:10.1046/j.1365-2249.2002.01974.x.

    Article  PubMed  CAS  Google Scholar 

  28. Tabatabaie P, Aghamohammadi A, Mamishi S, Isaeian A, Heidari G, Abdollahzade S, MirSaeid Ghazi B, Yeganeh M, Cheraghi T, Abolhasani H, Saghafi S, Alizadeh H, Anaraki MR, et al. Evaluation of humoral immune function in patients with bronchiectasis. Iran J Allergy Asthma Immunol. 2008;7(2):69–77.

    PubMed  CAS  Google Scholar 

  29. Stiehm RE. The four most common pediatric immunodeficiencies. Adv Exp Med Biol. 2007;601:15–26.

    PubMed  Google Scholar 

  30. Golshan M, Mohamad-Zadeh Z, Zahedi-Nejad N, Rostam-Poor B. Prevalence of asthma and related symptoms in primary school children of Isfahan, Iran, in 1998. Asian Pac J Allergy Immunol. 2001;19(3):163–70.

    PubMed  CAS  Google Scholar 

  31. Golshan M, Mohammad-Zadeh Z, Khanlar-Pour A, Iran-Pour R. Prevalence of asthma and related symptoms in junior high school children in Isfahan, Iran. Monaldi Arch Chest Dis. 2002;57(1):19–24.

    PubMed  CAS  Google Scholar 

  32. Miri S, Farid R, Akbari H, Amin R. Prevalence of allergic rhinitis and nasal smear eosinophilia in 11- to 15 yr-old children in Shiraz. Pediatr Allergy Immunol. 2006;17(7):519–23. doi:10.1111/j.1399-3038.2006.00424.x.

    Article  PubMed  Google Scholar 

  33. Mirsaid Ghazi B, Imamzadehgan R, Aghamohammadi A, Darakhshan Davari R, Rezaei N. Frequency of allergic rhinitis in school-age children (7–18 Years) in Tehran. Iran J Allergy Asthma Immunol. 2003;2(4):181–4.

    PubMed  Google Scholar 

  34. Ammann AJ, Hong R. Selective IgA deficiency: presentation of 30 cases and a review of the literature. Medicine 1971;60:223–36. doi:10.1097/00005792-197105000-00004.

    Article  Google Scholar 

  35. Woof JM, Kerr MA. The function of immunoglobulin A in immunity. J Pathol. 2006;208(2):270–82. doi:10.1002/path.1877.

    Article  PubMed  CAS  Google Scholar 

  36. Bjorkander J, Bake B, Oxelius VA, Hanson LA. Impaired lung function in patients with IgA deficiency and low levels of IgG2 or IgG3. N Engl J Med. 1985;313(12):720–4.

    PubMed  CAS  Google Scholar 

  37. Bossuyt X, Moens L, Van Hoeyveld E, et al. Coexistence of (partial) immune defects and risk of recurrent respiratory infections. Clin Chem. 2007;53(1):124–30. doi:10.1373/clinchem.2007.075861.

    Article  PubMed  CAS  Google Scholar 

  38. French MA, Denis KA, Dawkins R, Peter JB. Severity of infections in IgA deficiency: correlation with decreased serum antibodies to pneumococcal polysaccharides and decreased serum IgG2 and/or IgG4. Clin Exp Immunol. 1995;100(1):47–53.

    PubMed  CAS  Google Scholar 

  39. Oxelius VA, Laurell AB, Lindquist B, et al. IgG subclasses in selective IgA deficiency: importance of IgG2-IgA deficiency. N Engl J Med. 1981;304(24):1476–7.

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pediatrics, Division of Immunology and Allergy, Children Medical Center Hospital, Tehran University of Medical Sciences, 62 Gharib St, Keshavarz Blvd, 14194, Tehran, Iran

    Asghar Aghamohammadi, Taher Cheraghi, Mohammad Gharagozlou, Masoud Movahedi, Nima Rezaei, Nima Parvaneh, Hassan Abolhassani, Zahra Pourpak & Mostafa Moin

  2. Immunology, Asthma, and Allergy Research Institute, Children Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran

    Asghar Aghamohammadi, Mohammad Gharagozlou, Masoud Movahedi, Nima Rezaei, Mehdi Yeganeh, Zahra Pourpak & Mostafa Moin

  3. Growth and Development Research Center, Children Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran

    Asghar Aghamohammadi, Nima Parvaneh & Hassan Abolhassani

Authors
  1. Asghar Aghamohammadi
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Taher Cheraghi
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Mohammad Gharagozlou
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Masoud Movahedi
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Nima Rezaei
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Mehdi Yeganeh
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Nima Parvaneh
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Hassan Abolhassani
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Zahra Pourpak
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Mostafa Moin
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Asghar Aghamohammadi.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Aghamohammadi, A., Cheraghi, T., Gharagozlou, M. et al. IgA Deficiency: Correlation Between Clinical and Immunological Phenotypes. J Clin Immunol 29, 130–136 (2009). https://doi.org/10.1007/s10875-008-9229-9

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10875-008-9229-9

Keywords

Search

Navigation