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Atopic Manifestations Are Underestimated Clinical Features in Various Primary Immunodeficiency Disease Phenotypes

de Wit J1, Dalm VASH2,3*, Totté JEE1*, Kamphuis LSJ4, Vermont CL5, van Osnabrugge FY1, van Hagen PM2,3, Pasmans SGMA1,6 on behalf of the Academic Centers for Allergic Diseases and the Rare Immunological Disease Centre

1Department of Dermatology, Erasmus MC University Medical Center, Rotterdam, The Netherlands
2Department of Internal Medicine, Division of Clinical Immunology, Erasmus MC University Medical Center, Rotterdam, The Netherlands
3Department of Immunology, Erasmus MC University Medical Center, Rotterdam, The Netherlands
4Department of Pulmonary Medicine, Erasmus MC University Medical Center, Rotterdam, The Netherlands
5Department of Pediatrics, Division of Infectious Diseases and Immunology, Erasmus MC University Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands
6Department of Dermatology, Erasmus MC University Medical Center-Sophia Children's Hospital-Center of Pediatric Dermatology, Rotterdam, The Netherlands
*These authors contributed equally to this work.

J Investig Allergol Clin Immunol 2023; Vol 33(3) : 200-208
doi: 10.18176/jiaci.0768

Background: Atopic manifestations are described as a clinical feature of various primary immunodeficiency disease (PID) phenotypes and are frequently reported in combined immunodeficiencies. The prevalence of atopic manifestations in other PIDs remains largely unknown.
Objective: To evaluate the prevalence of atopic manifestations in PIDs other than combined immunodeficiencies and to identify in which PIDs atopic manifestations are most common with the aim of improving patient care.
Methods: A partner-controlled, questionnaire-based study was performed in pediatric and adult PID patients. Data from diagnostic tests to assess atopic manifestations (ie, diagnostic criteria for atopic dermatitis, spirometry, specific IgE against food and inhalant allergens) were collected from adult patients to confirm patient-reported atopic manifestations.
Results: Forty-seven children and 206 adults with PIDs and 56 partner-controls completed the questionnaire. Thirty-five pediatric patients (74.5%) and 164 adult patients (79.6%) reported having experienced 1 or more atopic manifestations compared with 28 partner-controls (50.0%). In the comparison of adult patients with partner-controls, prevalence values were as follows: atopic dermatitis, 49.5% vs 27.3% (P=.003); food allergy, 10.7% vs 1.9% (P=.031); asthma, 55.7% vs 14.8% (P<.001); and allergic rhinitis, 49.8% vs 21.8% (P<.001). The frequency of current atopic manifestations reported by patients was higher than the prevalence based on diagnostic tests (atopic dermatitis, 11.2%; food allergy, 1.9%; asthma 16.4%; and allergic rhinitis, 11.5%).
Conclusion: Atopic manifestations are prevalent clinical features across a broad spectrum of PIDs and, in our cohort, frequently present in patients with combined immunodeficiencies and predominant antibody deficiencies. Atopic manifestations should be evaluated in patients with PIDs.

Key words: Asthma, Atopic dermatitis, Food hypersensitivity, Immunologic deficiency syndromes, Seasonal allergic rhinitis