Atopic Disease in Primary Immune Deficiency with B Cell Dysfunction Amongst a U.S. Immunodeficiency Network (USIDNET) Cohort
Sunday, March 5, 2017
Exhibit Hall B2 (Georgia World Congress Center, Building B)
Gretchen A Harmon, MD, Magee L DeFelice, MD, Megan Morsheimer, MD, MPH
Rationale: Atopic disease is an important consideration in the care of patients with primary immune deficiencies (PID), however, forming the diagnosis may be challenging in patients with low or undetectable serum IgE. We aim to describe the prevalence of allergic disease in a cohort of patients with PID and B cell dysfunction, with the goal of improving recognition of such patients who may benefit from allergy treatment.

Methods: A query was submitted to the USIDNET database to examine associated conditions, allergic reactions, and use of allergen immunotherapy in patients with a diagnosis of a PID with B cell dysfunction.

Results: The query identified 2391 cases, the majority (63%) with a diagnosis of common variable immunodeficiency disorders with unknown genetic basis (CVID). Five patients received immunotherapy. For each PID within this cohort, allergic rhinitis and asthma were reported in the following percentages: Activated PI3Kd (0%, 0%), agammaglobulinemia of unknown cause or unlisted gene defect (10%, 20%), AID deficiency (0%, 33%), BTK deficiency (2%, 9%), CD40L deficiency (0%, 4%), CVID (5%, 29%), NEMO deficiency (11%, 26%), Hyper IgM due to uncertain or unlisted cause (<1%, 3%), hypogammaglobulinemia of unknown cause or unlisted gene defect (<1%, 28%), isolated IgG subclass deficiency (7%, 53%), selective IgA deficiency (6%, 45%), specific antibody deficiency with normal IgG concentrations and normal numbers of B cells (11%, 52%), TACI deficiency (60%, 20%), transient hypogammaglobulinemia of infancy with normal numbers of B cells (11%, 33%).

Conclusions: The data suggest that atopic disease is not uncommonly seen in the setting of B cell dysfunction.