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Patients with immunodeficiencies and antibody deficiencies typically have atopic symptoms, which should be addressed to avoid worsening of the condition.

Primary immunodeficiency diseases (PIDs) are a heterogeneous set of more than 300 inherited immunity abnormalities caused by variations in genes encoding functional proteins of human immune cells. The estimated incidence of symptomatic PIDs is 1 in 2,000 live newborns, while the frequency is 1 in 10,000–12,000 in the general population. In most cases, PIDs are characterized by repeated and/or severe infections. Patients may also have autoimmunity, autoinflammation, cancer, and allergy diseases. The so-called atopic syndrome, defined by atopic dermatitis (AD), food allergy (FA), asthma, and allergic rhinitis, may include allergic symptoms.

Atopic symptoms are listed as a clinical trait of several PID phenotypes, with mixed immunodeficiencies, in particular, being frequently documented. Data on atopic symptoms in other PIDs are limited.

This study, published in the Journal of Investigational Allergology and Clinical Immunology, aimed to determine the prevalence of atopic manifestations in different PIDs.

A questionnaire-based study with partner control was conducted in pediatric and adult PID patients. To confirm the patient’s reports of atopic manifestations, data from diagnostic tests for atopic manifestations (such as diagnostic criteria for AD, spirometry, and specific IgE against food and inhalant allergens) were gathered in adult patients.

The questionnaire was completed by 47 PID-affected children, 206 PID-affected adults, and 56 partner-control participants. Compared to 28 (50.0%) partner-controls, 35 (74.5%) pediatric and 164 (79.6%) adult patients reported having ever experienced one or more atopic symptoms. Atopic dermatitis prevalence in adult patients compared to partner-controls was 49.5% vs. 27.3% (p=0.003), food allergy prevalence was 10.7% vs. 1.9% (p=0.031), asthma prevalence was 55.7% vs. 14.8% (p<0.001), and allergic rhinitis prevalence was 49.8% vs. 21.8% (p<0.001). Atopic dermatitis, food allergies, asthma, and allergic rhinitis all had greater reported occurrences of present atopic symptoms than predicted by diagnostic testing (atopic dermatitis 11.2%, food allergies 1.9%, asthma 16.4%, and allergic rhinitis 11.5%).

This questionnaire-based study concluded that patients with combined immunodeficiencies (CIDs) and predominant antibody deficiencies (PADs) were more likely to have atopic symptoms. To avoid worsening of the patient’s condition, it is recommended to assess patients with CIDs and PADs for atopic symptoms. Future research should focus on finding unique characteristics of atopic manifestations in PIDs that may facilitate recognizing the underlying PID. 

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Reference:

de Wit, J., Dalm, V., Totté, J. E., Kamphuis, L. S., Vermont, C. L., van Osnabrugge, F. Y., van Hagen, P. M., & Pasmans, S. G. (2021). Atopic manifestations are underestimated clinical features in various primary immunodeficiency disease phenotypes. Journal of Investigational Allergology and Clinical Immunology, 0. https://doi.org/10.18176/jiaci.0768