To investigate the clinical and laboratory features of children with selective immunoglobulin (Ig)A deficiency followed in a tertiary children’s hospital.
The medical records of 118 children (63 boys, 55 girls), median age 7 years (range 4–18 years), seen over a 5-year period were retrospectively reviewed.
Patients with IgA levels <7 g/L and normal or elevated IgG and IgM were included in this study. Medical records were reviewed and laboratory studies included quantitative immunoglobulins, IgG subclasses, IgE, and autoantibodies. Medical history was evaluated for evidence of infections, allergic disease, and autoimmunity.
Sixty-one percent of these patients had been followed for >6 months, with 99 (84%) of the 118 patients with a history of recurrent infections, primarily upper respiratory infections. In addition, 51 (43%) of 118 had a history of allergic disorders, including predominantly rhinitis, asthma, and atopic dermatitis. Finally, 20 (17%) of 118 had a history of autoimmune disease with a wide range of disorders, including celiac disease and type 1 diabetes being the most prevalent.
Children with selective IgA deficiency may be more prone to upper respiratory infections, allergic disease, and autoimmunity.
This report focuses on children with selective IgA deficiency referred to a tertiary children’s hospital, so it suffers from a potential for significant referral bias. However, in light of another recent publication on selective IgA deficiency (Jorgensen GH, et al. Clinical symptoms in adults with selective IgA deficiency: a case-control study. J Clin Immunol 2013;33:742–747), there is a real potential that selective IgA deficiency is associated with a greater degree of clinically significant symptomatology than has previously been appreciated. Importantly, in some cases these include recurrent sinopulmonary infections with encapsulated bacteria, whereas in others it is less clear while the incidence of allergic disease and autoimmunity is significantly higher. These observations appear to suggest that secretory IgA alone plays a less significant role in protecting the mucosal surfaces from bacterial infection while it may have a more prominent role in protecting the immune system at the level of the mucosa from interactions that increase the incidence of allergic disease and autoimmunity. There are no registries for selective IgA deficiency and the bulk of published studies from the past contend that the overwhelming majority of affected individuals are asymptomatic. Because of selection bias and/or small sample size in most reports to date, resolving the actual impact of selective IgA deficiency awaits studies in which ascertainment bias is dealt with and appropriate case controls are included together with long-term follow-up.
- Copyright © 2013 by the American Academy of Pediatrics