What should pediatrician know about selective IgA deficiency (update 2017)

O.L. Logvinova, M.O. Gonchar, O.P. Pomazunovska


The article presents analysis of the current state of selective immunoglobulin A deficiency (SIgAD) from the point of view of the world’s leading experts, based on the results of epidemiological, genetic, molecular studies with high level of evidence. Selective IgA deficiency is the most common primary immunodeficiency and is characterized by an isolated deficiency of IgA, with usually normal levels of IgM and IgG in patients older than 4 years. Some IgG subclasses may be reduced in some patients with IgA deficiency. It is assumed that the overall incidence of selective IgA deficiency is 1 : 500–1 : 2000, but may be higher as most SIgADs have asymptomatic course, and there is no IgA deficiency screening program. IgA deficiency is facilitated by a complex network of genetic effects, namely IFIH1 loci on chromosome 2 and HLA-DQA1 on chromosome 6, with IgA-deficient-linked PVT1 (8 chromosomes), ATG13-AMBRA1 (11 chromosomes), AHI1 (6 chromosomes), and CLEC16A (16 chromosomes), including genes that are known to affect the physiology of IgA production. Most people with SIgAD are asymptomatic carriers, for some patients, pulmonary infections, allergies, autoimmune diseases, gastrointestinal diseases and malignant neoplasms are characteristic. Monitoring of patients with SIgAD consists of various methods, including education, the treatment of allergic or autoimmune diseases, the use of long courses or even the prophylactic use of antibiotics, the use of polyvalent pneumococcal vaccines and intravenous or subcutaneous substitution therapy with immunoglobulins. SIgAD prognosis mainly depends on the phenotype. There have been rare cases of spontaneous recovery, especially in young patients. Rarely, selective IgA deficiency may progress to the overall variable immunodeficiency. We consider it expedient to use the world experience in the diagnosis and treatment of selective IgA deficiency as the basis for patients’ monitoring in Ukraine.


selective IgA deficiency; diagnosis; prevention of complications


Chernyshova LI, Volokha AP, Lapiy FI. Immunological basis of immunization. Zdorov'ye Rebenka. 2014;1(52):188-92. (in Ukrainian).

Immunoglobulin A (IgA). Available from: Accessed: September 10, 2010.

Yazdani R., Azizi G., Abolhassani H, Aghamohammadi A. Selective IgA Deficiency: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management. Scand J Immunol. 2017 Jan;85(1):3-12. doi: 10.1111/sji.12499.

Yazdani R, Latif A, Tabassomi F, et al. Clinical phenotype classification for selective immunoglobulin A deficiency. Expert Rev Clin Immunol. 2015;11(11):1245-54. doi: 10.1586/1744666X.2015.1081565.

Pallav K, Xu K, Leffle DA, Kabbani T, Kelly CP. Immunoglobulin A deficiency in celiac disease in the United States. J Gastroenterol Hepatol. 2016 Jan; 31(1): 133-7. doi: 10.1111/jgh.13176.

Bronson PG, Chang D, Bhangale T, et al. Common variants at PVT1, ATG13-AMBRA1, AHI1 and CLEC16A are associated with selective IgA deficiency. Nat Genet. 2016 Nov;48(11):1425-9. doi: 10.1038/ng.3675.

Mertin S, Thomson I. What you need to know about IgA deficiency: a case study. J Am Assoc Nurse Pract. 2014 May;26(5):268-72. doi: 10.1002/2327-6924.12077.

Cipe F, Doðu F, Güloðlu D, et al. B-cell subsets in patients with transient hypogammaglobulinemia of infancy, partial IgA deficiency, and selective IgM deficiency. J Investig Allergol Clin Immunol. 2013;23(2):94-100.

Maruyama S, Okamoto Y, Toyoshima M, Hanaya R, Kawano Y. Immunoglobulin A deficiency following treatment with lamotrigine . Brain Dev. 2016 Nov;38(10):947-949. doi: 10.1016/j.braindev.2016.06.006.

Yazdani R, Fatholahi M, Ganjalikhani-Hakemi M, et al. Role of apoptosis in common variable immunodeficiency and selective immunoglobulin A deficiency. Mol Immunol. 2016 Mar;71:1-9. doi: 10.1016/j.molimm.2015.12.016.

Azizi G, Ziaee V, Tavakol M, et al. Approach to the Management of Autoimmunity in Primary Immunodeficiency. Scand J Immunol. 2017 Jan;85(1):13-29. doi: 10.1111/sji.12506.

Jolles S, Chapel H, Litzman J. When to initiate immunoglobulin replacement therapy (IGRT) in antibody deficiency: a practical approach. Clinical and Experimental Immunology. 188(3):333-41. doi: 10.1111/cei.12915.

Shkalim V, Monselize Y, Segal N, Zan-Bar I, Hoffer V, Garty BZ. Selective IgA deficiency in children in Israel. J Clin Immunol. 2010 Sep;30(5):761-5. doi: 10.1007/s10875-010-9438-x.

Bonilla FA, Khan DA, Ballas ZK, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allergy Clin Immunol. 2015 Nov;136(5):1186-205.e1-78. doi: 10.1016/j.jaci.2015.04.049.

Milito C, Pulvirenti F, Pesce AM, et al. 2014.Adequate patient's outcome achieved with short immunoglobulin replacement intervals in severe antibody deficiencies. J Clin Immunol. 2014;34(7):813-9.

Berger M, Jolles S, Orange JS, Sleasman JW. Bioavailability of IgG administered by the subcutaneous route. J Clin Immunol. 2013 Jul;33(5):984-90. doi: 10.1007/s10875-013-9876-3.

Cheraghi T, Aghamohammadi A, Mirminachi B, et al. Prediction of the evolution of common variable immunodeficiency: HLA typing for patients with selective IgA deficiency. J Investig Allergol Clin Immunol. 2014;24(3):198-200. PMID: 25011360.

Copyright (c) 2017 Zdorov'ye Rebenka - Child`s Health

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.


© Publishing House Zaslavsky, 1997-2019


   Seo анализ сайта