Prevalent Autoimmunities in Patients with Selective IgA Deficiency
Abstract
Background/objectives: Among primary immunodeficiency (PID), selective immunoglobulin A deficiency (SIgAD) is the most prevalent type. SIgAD patients can be either asymptomatic or symptomatic. Symptomatic patients suffer from a wide range of manifestations including infections, allergy, autoimmunity, and malignancy. SIgAD patients are more susceptible to some autoimmune diseases, while the exact mechanisms behind this association are not found yet. Therefore, this study was conducted in order to evaluate the possible association between autoimmune disease and specific clinical records or immunological data in SIgAD patients.
Methods: The present cohort included 166 SIgAD patients who were diagnosed at the Research Centre for Immunodeficiencies at the children’s medical Centre. A comprehensive history, demographic information, clinical manifestations, laboratory data were obtained from all patients to assess the autoimmune complications.
Results: Autoimmunity was seen in 16 patients (9.6%, 10 males and 6 females). The most common autoimmunity types were juvenile idiopathic arthritis, vitiligo and alopecia (18.8%). 9 patients (6.5%) had a PID family history. Significant data that were higher in patients with autoimmunity were the mean age at the study time (p=0.019), rheumatoid problem (p=0.043), liver problem (p=0.031), IgG level (p=0.006) and IgE level (p=0.004).
Conclusion: The association between SIgA deficiency and autoimmunity could lead to severe clinical complications. So, it is better for immunologists to aware of these problems.
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2. Borte S, Pan-Hammarström Q, Liu C, Sack U, Borte M, Wagner U, et al. Interleukin-21 restores immunoglobulin production ex vivo in patients with common variable immunodeficiency and selective IgA deficiency. 2009;114(19):4089-98.
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7. Edwards E, Razvi S, Cunningham-Rundles CJCi. IgA deficiency: clinical correlates and responses to pneumococcal vaccine. 2004;111(1):93-7.
8. 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. 2009;133(1):78-85.
9. Wang N, Shen N, Vyse TJ, Anand V, Gunnarson I, Sturfelt G, et al. Selective IgA deficiency in autoimmune diseases. 2011;17(11-12):1383-96.
10. Jorgensen G, Gardulf A, Sigurdsson M, Sigurdardottir ST, Thorsteinsdottir I, Gudmundsson S, et al. Clinical symptoms inadults with selective IgA deficiency: a casecontrol study. 2013;33(4):742-7.
11. Yazdani R, Azizi G, Abolhassani H, Aghamohammadi AJSjoi. Selective IgA deficiency: epidemiology, pathogenesis, clinical phenotype, diagnosis, prognosis and management. 2017;85(1):3-12.
12. Aghamohammadi A, Mohammadi J, Parvaneh N, Rezaei N, Moin M, Espanol T, et al. Progression of selective IgA deficiency to common variable immunodeficiency. 2008;147(2):87-92.
13. Hammarström L, Vorechovsky I, Webster DJC, Immunology E. Selective IgA deficiency (SIgAD) and common variable immunodeficiency (CVID). 2000;120(2):225-31.
14. Aghamohammadi A, Cheraghi T, Gharagozlou M, Movahedi M, Rezaei N, Yeganeh M, et al. IgA deficiency: correlation between clinical and immunological phenotypes. 2009;29(1):130-6.
15. Cunningham-Rundles CJJoci. Physiology of IgA and IgA deficiency. 2001;21(5):303- 9.
16. Jacob CM, Pastorino AC, Fahl K, Carneiro-Sampaio M, Monteiro RCJJoci. Autoimmunity in IgA deficiency: revisiting the role of IgA as a silent housekeeper. 2008;28(1):56-61.
17. Stiehm ERJJoi. The four most common pediatric immunodeficiencies. 2008;5(2):227-34.
18. Arkwright PD, Abinun M, Cant AJJB. Autoimmunity in human primary immunodeficiency diseases. 2002;99(8):2694-702.
19. Soheili H, Abolhassani H, Arandi N, Khazaei HA, Shahinpour S, HirbodMobarakeh A, et al. Evaluation of natural regulatory T cells in subjects with selective IgA deficiency: from senior idea to novel opportunities. 2013;160(2):208-14.
20. Yazdani R, Ganjalikhani-Hakemi M, Esmaeili M, Abolhassani H, Vaeli S, Rezaei A, et al. Impaired Akt phosphorylation in Bcells of patients with common variable immunodeficiency. 2017;175:124-32.
21. Jorgensen GH, Thorsteinsdottir I, Gudmundsson S, Hammarstrom L, Ludviksson BR. Familial aggregation of IgAD and autoimmunity. Clinical immunology. 2009;131(2):233-9.
22. Vale AM, Schroeder Jr HW. Clinical consequences of defects in B-cell development. Journal of Allergy and Clinical Immunology. 2010;125(4):778-87.
23. Russell M, Sibley D, Nikolova E, Tomana M, Mestecky J. IgA antibody as a noninflammatory regulator of immunity. Portland Press Limited; 1997.
24. Abolhassani H, Gharib B, Shahinpour S, Masoom S, Havaei A, Mirminachi B, et al. Autoimmunity in patients with selective IgA deficiency. Journal of investigational allergology & clinical immunology. 2015:25(2):112-9.25. Etzioni A. Immune deficiency and autoimmunity. Autoimmunity reviews. 2003;2(6):364 -9.
26. Cooper GS, Bynum ML, Somers EC. Recent insights in the epidemiology of autoimmune diseases: improved prevalence estimates and understanding of clustering of diseases. Journal of autoimmunity. 2009;33(3 -4):197 -207.
2. Borte S, Pan-Hammarström Q, Liu C, Sack U, Borte M, Wagner U, et al. Interleukin-21 restores immunoglobulin production ex vivo in patients with common variable immunodeficiency and selective IgA deficiency. 2009;114(19):4089-98.
3. Okahashi N, Yamamoto M, Vancott JL, Chatfield SN, Roberts M, Bluethmann H, et al. Oral immunization of interleukin-4 (IL-4) knockout mice with a recombinant Salmonella strain or cholera toxin reveals that CD4+ Th2 cells producing IL-6 and IL10 are associated with mucosal immunoglobulin A responses. 1996;64(5):1516-25.
4. Ramsay AJ, Husband AJ, Ramshaw IA, Bao S, Matthaei KI, Koehler G, et al. The role of interleukin-6 in mucosal IgA antibody responses in vivo. 1994;264(5158):561-3.
5. Yazdani R, Latif A, Tabassomi F, Abolhassani H, Azizi G, Rezaei N, et al. Clinical phenotype classification for selective immunoglobulin A deficiency. Expert review of clinical immunology. 2015;11(11):1245-54. Epub 2015/08/27.
6. Ammann AJ, HONG RJM. Selective IgA deficiency: presentation of 30 cases and a review of the literature. 1971;50(3):223.
7. Edwards E, Razvi S, Cunningham-Rundles CJCi. IgA deficiency: clinical correlates and responses to pneumococcal vaccine. 2004;111(1):93-7.
8. 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. 2009;133(1):78-85.
9. Wang N, Shen N, Vyse TJ, Anand V, Gunnarson I, Sturfelt G, et al. Selective IgA deficiency in autoimmune diseases. 2011;17(11-12):1383-96.
10. Jorgensen G, Gardulf A, Sigurdsson M, Sigurdardottir ST, Thorsteinsdottir I, Gudmundsson S, et al. Clinical symptoms inadults with selective IgA deficiency: a casecontrol study. 2013;33(4):742-7.
11. Yazdani R, Azizi G, Abolhassani H, Aghamohammadi AJSjoi. Selective IgA deficiency: epidemiology, pathogenesis, clinical phenotype, diagnosis, prognosis and management. 2017;85(1):3-12.
12. Aghamohammadi A, Mohammadi J, Parvaneh N, Rezaei N, Moin M, Espanol T, et al. Progression of selective IgA deficiency to common variable immunodeficiency. 2008;147(2):87-92.
13. Hammarström L, Vorechovsky I, Webster DJC, Immunology E. Selective IgA deficiency (SIgAD) and common variable immunodeficiency (CVID). 2000;120(2):225-31.
14. Aghamohammadi A, Cheraghi T, Gharagozlou M, Movahedi M, Rezaei N, Yeganeh M, et al. IgA deficiency: correlation between clinical and immunological phenotypes. 2009;29(1):130-6.
15. Cunningham-Rundles CJJoci. Physiology of IgA and IgA deficiency. 2001;21(5):303- 9.
16. Jacob CM, Pastorino AC, Fahl K, Carneiro-Sampaio M, Monteiro RCJJoci. Autoimmunity in IgA deficiency: revisiting the role of IgA as a silent housekeeper. 2008;28(1):56-61.
17. Stiehm ERJJoi. The four most common pediatric immunodeficiencies. 2008;5(2):227-34.
18. Arkwright PD, Abinun M, Cant AJJB. Autoimmunity in human primary immunodeficiency diseases. 2002;99(8):2694-702.
19. Soheili H, Abolhassani H, Arandi N, Khazaei HA, Shahinpour S, HirbodMobarakeh A, et al. Evaluation of natural regulatory T cells in subjects with selective IgA deficiency: from senior idea to novel opportunities. 2013;160(2):208-14.
20. Yazdani R, Ganjalikhani-Hakemi M, Esmaeili M, Abolhassani H, Vaeli S, Rezaei A, et al. Impaired Akt phosphorylation in Bcells of patients with common variable immunodeficiency. 2017;175:124-32.
21. Jorgensen GH, Thorsteinsdottir I, Gudmundsson S, Hammarstrom L, Ludviksson BR. Familial aggregation of IgAD and autoimmunity. Clinical immunology. 2009;131(2):233-9.
22. Vale AM, Schroeder Jr HW. Clinical consequences of defects in B-cell development. Journal of Allergy and Clinical Immunology. 2010;125(4):778-87.
23. Russell M, Sibley D, Nikolova E, Tomana M, Mestecky J. IgA antibody as a noninflammatory regulator of immunity. Portland Press Limited; 1997.
24. Abolhassani H, Gharib B, Shahinpour S, Masoom S, Havaei A, Mirminachi B, et al. Autoimmunity in patients with selective IgA deficiency. Journal of investigational allergology & clinical immunology. 2015:25(2):112-9.25. Etzioni A. Immune deficiency and autoimmunity. Autoimmunity reviews. 2003;2(6):364 -9.
26. Cooper GS, Bynum ML, Somers EC. Recent insights in the epidemiology of autoimmune diseases: improved prevalence estimates and understanding of clustering of diseases. Journal of autoimmunity. 2009;33(3 -4):197 -207.
| Files | ||
| Issue | Vol 2, No 3 (2019) | |
| Section | Original Article | |
| DOI | https://doi.org/10.22034/igj.2019.200805.1023 | |
| Keywords | ||
| Agammaglobulinemia Autoimmunity Primary immunodeficiency disorders(PIDs) SIgAD | ||
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How to Cite
1.
Pashangzadeh S, Sohani M. Prevalent Autoimmunities in Patients with Selective IgA Deficiency. Immunol Genet J. 2019;2(3):135-146.
