Prevalence of Atopic Diseases in Patients with Humoral Primary Immunodeficiency: A Comparison of a Single Center and the US Immunodeficiency Network (USIDNET)
Saturday, March 5, 2016
South Exhibit Hall H (Convention Center)
Alice S. Chau, MD, Artemio M. Jongco III, MD PhD MPH, Laura Helfner, MD, James C. Fagin, MD, Vincent R. Bonagura, MD FAAAAI
Rationale: Humoral primary immunodeficiency diseases (PIDDs) arise from defects in B cell differentiation, maturation, and class switch recombination.  Nevertheless, a subset of these patients demonstrate atopic disease with corresponding elevated IgE.  We characterized the prevalence and kinds of atopic conditions present in two distinct populations.

Methods: We determined the prevalence of IgE-mediated atopic disease and immunologic characteristics of humoral PIDD patients seen at an academic medical center immunology clinic (retrospective chart review, 2001-2011) and enrolled in the USIDNET Registry (queried 5/5/2015).  The following diagnoses were included: congenital agammaglobulinemia (279.04), idiopathic agammaglobulinemia (279.00), selective IgA deficiency (279.01), IgG subclass deficiency (279.03), specific antibody deficiency (279.19), and hyper IgM syndromes (279.05).

Results: Sixty-Five of 92 clinic patients and 616 of 4087 registry participants were included in the analysis.  The distribution of PIDD diagnoses was as follows (clinic;USIDNET): 279.00(32.0%;2.3%), 279.01(22.0%;4.5%), 279.03(0%;1.9%), 279.04(20.0%;61.7%), 279.05(3.0%;23.9%), and 279.19(23.0%;5.8%).  The distribution of atopic diagnoses were as follows: allergic rhinitis(38.5%;8.6%), asthma(28.6%;43.6%), drug allergy(11.0%;28.8%), eczema(6.6;16.5%), and food allergy(2.2%;2.5%).  ImmunoCAP or skin prick testing and serum IgE values were available in 41 and 55 clinic patients, respectively, while serum IgE was documented in 131 database patients.  In both cohorts, 279.04 and 279.05 patients had the most number of undetectable IgE.

Conclusions: Humoral PIDD patients can develop IgE-mediated type 1 hypersensitivities, many with documented serum IgE levels.  The distribution of PIDD and atopic diagnoses differ between the two populations.  Both registry and single center data are informative and do not necessarily demonstrate similar trends.