Skip to main content
Top
Published in: Journal of Clinical Immunology 1/2015

01-01-2015 | Brief Communication

Reversal of Immunoglobulin A Deficiency in Children

Authors: Che Kang Lim, Charlotte Dahle, Kerstin Elvin, Bengt A. Andersson, Johan Rönnelid, Erik Melén, Anna Bergström, Lennart Truedsson, Lennart Hammarström

Published in: Journal of Clinical Immunology | Issue 1/2015

Login to get access

Abstract

Purpose

Immunoglobulin A deficiency (IgAD) is the most common primary immunodeficiency in the general population. It is defined as a serum IgA level below or equal to 0.07 g/l with normal IgM and IgG levels in children over the age of 4. However, a few cases of reversal of IgAD at later ages have been observed previously, especially in pediatric patients. This study aimed at investigating the frequency of reversal in a large cohort of children and young adults in order to evaluate the present definition of IgAD.

Methods

Clinical laboratory records from 654 pediatric IgA deficient patients, 4–13 years of age, were retrieved from five university hospitals in Sweden. Follow up in the children where IgA serum levels had been routinely measured was subsequently performed. In addition, follow up of the IgA-levels was also performed at 4, 8 and 16 years of age in children who were IgA deficient at the age of 4 years in a Swedish population-based birth cohort study in Stockholm (BAMSE).

Results

Nine out of 39 (23.1 %) children who were identified as IgAD at 4 years of age subsequently increased their serum IgA level above 0.07 g/L. The average age of reversal was 9.53 ± 2.91 years. In addition, 30 out of the 131 (22.9 %) children with serum IgAD when sampled between 5 and 9.99 years of age reversed their serum IgA level with time. The BAMSE follow up study showed a reversal of IgAD noted at 4 years of age in 8 out of 14 IgAD children at 16 years of age (5 at 8 years of age) where 4 were normalized their serum IgA levels while 4 still showed low serum levels of IgA, yet above the level defining IgAD. The results indicate that using 4 years of age, as a cut off for a diagnosis of IgAD may not be appropriate.

Conclusions

Our findings suggest that a diagnosis of IgAD should not be made before the early teens using 0.07 g/L of IgA in serum as a cut off.
Appendix
Available only for authorised users
Literature
1.
go back to reference 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.PubMedCrossRef 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.PubMedCrossRef
2.
go back to reference Al-Herz W, Bousfiha A, Casanova JL, Chapel H, Conley ME, Cunningham-Rundles C, et al. Primary immunodeficiency diseases: an update on the classification from the international union of immunological societies expert committee for primary immunodeficiency. Front Immunol. 2011;2:54. doi:10.3389/fimmu.2011.00054.PubMedCentralPubMedCrossRef Al-Herz W, Bousfiha A, Casanova JL, Chapel H, Conley ME, Cunningham-Rundles C, et al. Primary immunodeficiency diseases: an update on the classification from the international union of immunological societies expert committee for primary immunodeficiency. Front Immunol. 2011;2:54. doi:10.​3389/​fimmu.​2011.​00054.PubMedCentralPubMedCrossRef
3.
go back to reference Koskinen S. Long-term follow-up of health in blood donors with primary selective IgA deficiency. J Clin Immunol. 1996;16(3):165–70.PubMedCrossRef Koskinen S. Long-term follow-up of health in blood donors with primary selective IgA deficiency. J Clin Immunol. 1996;16(3):165–70.PubMedCrossRef
4.
go back to reference Rankin EC, Isenberg DA. IgA deficiency and SLE: prevalence in a clinic population and a review of the literature. Lupus. 1997;6(4):390–4.PubMedCrossRef Rankin EC, Isenberg DA. IgA deficiency and SLE: prevalence in a clinic population and a review of the literature. Lupus. 1997;6(4):390–4.PubMedCrossRef
5.
go back to reference Iizuka M, Itou H, Sato M, Yukawa M, Shirasaka T, Chiba M, et al. Crohn’s disease associated with selective immunoglobulin a deficiency. J Gastroenterol Hepatol. 2001;16(8):951–2.PubMedCrossRef Iizuka M, Itou H, Sato M, Yukawa M, Shirasaka T, Chiba M, et al. Crohn’s disease associated with selective immunoglobulin a deficiency. J Gastroenterol Hepatol. 2001;16(8):951–2.PubMedCrossRef
6.
go back to reference Hammarstrom L, Vorechovsky I, Webster D. Selective IgA deficiency (SIgAD) and common variable immunodeficiency (CVID). Clin Exp Immunol. 2000;120(2):225–31.PubMedCentralPubMedCrossRef Hammarstrom L, Vorechovsky I, Webster D. Selective IgA deficiency (SIgAD) and common variable immunodeficiency (CVID). Clin Exp Immunol. 2000;120(2):225–31.PubMedCentralPubMedCrossRef
7.
go back to reference Koskinen S, Tolo H, Hirvonen M, Koistinen J. Long-term persistence of selective IgA deficiency in healthy adults. J Clin Immunol. 1994;14(2):116–9.PubMedCrossRef Koskinen S, Tolo H, Hirvonen M, Koistinen J. Long-term persistence of selective IgA deficiency in healthy adults. J Clin Immunol. 1994;14(2):116–9.PubMedCrossRef
8.
go back to reference Buckley RH. Clinical and immunologic features of selective IgA deficiency. Birth Defects Orig Artic Ser. 1975;11(1):134–42.PubMed Buckley RH. Clinical and immunologic features of selective IgA deficiency. Birth Defects Orig Artic Ser. 1975;11(1):134–42.PubMed
9.
go back to reference Ostergaard PA. Clinical and immunological features of transient IgA deficiency in children. Clin Exp Immunol. 1980;40(3):561–5.PubMedCentralPubMed Ostergaard PA. Clinical and immunological features of transient IgA deficiency in children. Clin Exp Immunol. 1980;40(3):561–5.PubMedCentralPubMed
10.
go back to reference Plebani A, Ugazio AG, Monafo V, Burgio GR. Clinical heterogeneity and reversibility of selective immunoglobulin A deficiency in 80 children. Lancet. 1986;1(8485):829–31.PubMedCrossRef Plebani A, Ugazio AG, Monafo V, Burgio GR. Clinical heterogeneity and reversibility of selective immunoglobulin A deficiency in 80 children. Lancet. 1986;1(8485):829–31.PubMedCrossRef
11.
go back to reference de Laat PC, Weemaes CM, Bakkeren JA. Immunoglobulin levels during follow-up of children with selective IgA deficiency. Scand J Immunol. 1992;35(6):719–25.PubMedCrossRef de Laat PC, Weemaes CM, Bakkeren JA. Immunoglobulin levels during follow-up of children with selective IgA deficiency. Scand J Immunol. 1992;35(6):719–25.PubMedCrossRef
13.
go back to reference Ekstrom S, Magnusson J, Kull I, Lind T, Almqvist C, Melen E, et al. Maternal BMI in early pregnancy and offspring asthma, rhinitis and eczema up to 16 years of age. Clin Exp Allergy J Br Soc Allergy Clin Immunol. 2014. doi:10.1111/cea.12340. Ekstrom S, Magnusson J, Kull I, Lind T, Almqvist C, Melen E, et al. Maternal BMI in early pregnancy and offspring asthma, rhinitis and eczema up to 16 years of age. Clin Exp Allergy J Br Soc Allergy Clin Immunol. 2014. doi:10.​1111/​cea.​12340.
15.
go back to reference Johnson ML, Keeton LG, Zhu ZB, Volanakis JE, Cooper MD, Schroeder Jr HW. Age-related changes in serum immunoglobulins in patients with familial IgA deficiency and common variable immunodeficiency (CVID). Clin Exp Immunol. 1997;108(3):477–83.PubMedCentralPubMedCrossRef Johnson ML, Keeton LG, Zhu ZB, Volanakis JE, Cooper MD, Schroeder Jr HW. Age-related changes in serum immunoglobulins in patients with familial IgA deficiency and common variable immunodeficiency (CVID). Clin Exp Immunol. 1997;108(3):477–83.PubMedCentralPubMedCrossRef
Metadata
Title
Reversal of Immunoglobulin A Deficiency in Children
Authors
Che Kang Lim
Charlotte Dahle
Kerstin Elvin
Bengt A. Andersson
Johan Rönnelid
Erik Melén
Anna Bergström
Lennart Truedsson
Lennart Hammarström
Publication date
01-01-2015
Publisher
Springer US
Published in
Journal of Clinical Immunology / Issue 1/2015
Print ISSN: 0271-9142
Electronic ISSN: 1573-2592
DOI
https://doi.org/10.1007/s10875-014-0112-6

Other articles of this Issue 1/2015

Journal of Clinical Immunology 1/2015 Go to the issue