The Allergic Phenotype of Children and Adolescents with Selective IgA Deficiency: A Longitudinal Monocentric Study

Abstract

Background: Selective IgA deficiency (SIgAD) is the most common inborn error of immunity. The exact prevalence and pathogenesis of allergy in SIgAD have not yet been defined. We aimed to describe the prevalence and the characteristics of allergy in pediatric SIgAD subjects, evaluate the association between allergy and other comorbidities, and define the immune phenotype of allergic and non-allergic patients. Methods: Clinical and immunological data from 67 SIgAD patients were collected over a 13-year period at a single center. Patients’ characteristics were analyzed according to the presence of allergy. Results: Allergy was diagnosed in 34% of SIgAD patients, with a median age at allergy diagnosis of 8 years. Allergy was the second-most-common clinical manifestation, following recurrent respiratory infections. Among the allergic group, 74% had rhinitis, 30% asthma, 30% atopic dermatitis, and 22% food allergy; one out of three had more than one allergic manifestation. SIgAD patients showed more frequent transitory lymphopenia and a lower count of CD19+ at diagnosis than at last FU. However, compared to non-allergic subjects, allergic patients did not differ in their immune phenotype, number and severity of infections, or increased autoimmunity. Conclusions: In our longitudinal study, compared to non-allergic SIgAD patients, those with allergies did not present a more severe immune defect or complex clinical phenotype. However, evaluation and early identification of allergy in the context of SIgAD assessment, both at diagnosis and during FU, and definition of a proper management are important to prevent complications and improve the patient’s quality of life.

Clinical and experimental treatment of primary humoral immunodeficiencies

“…Management of the allergies associated with sIgAD is similar to those in general. Apart from medications it is possible to use also immunotherapy and omalizumab [ 98 ]. Patients with sIgAD are more susceptible to autoimmune diseases (SLE, rheumatoid arthritis, thyroiditis, autoimmune hemolytic anemia, type 1 diabetes mellitus, Graves` disease, Crohn`s disease, ulcerative colitis, coeliac disease, autoimmune hepatitis, scleroderma, vitiligo, immune thrombocytopenic purpura, and autoimmune hemolytic anemia).…”

Allergy and autoimmunity in children: non-mutually exclusive diseases. A narrative review

“…Cinicola et al showed that patients with IgA deficiency and allergies do not have a complex immune defect, except for transient mild lymphopenia and low count CD19 + at diagnosis vs. follow-up (65% vs. 1.5%, p < 0.0001% and 57% vs. 11%, p < 0.0001, respectively) (65).…”

Reply to Özdemir, Ö. Allergic Disease with Selective IgA Deficiency. Comment on “Cinicola et al. The Allergic Phenotype of Children and Adolescents with Selective IgA Deficiency: A Longitudinal Monocentric Study. J. Clin. Med. 2022, 11, 5705”

“…We carefully read the correspondence [1] on our manuscript "The Allergic Phenotype of Children and Adolescents with Selective IgA Deficiency: A Longitudinal Monocentric Study" [2].…”