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Background: Atopic manifestations are describedas clinical feature of variousprimary immunodeficiency disease (PID) phenotypes and in particular frequently reported in the combined immune deficiencies. The prevalence of atopic manifestations in other PIDs remains largely unknown. Objective: To evaluate the prevalence of atopic manifestations in other PIDs and to identify in which PIDsatopic manifestations are most common in order to improve patient care.  Methods: A partner-controlled questionnaire-based study was performed in pediatric and adult PID patients. Subsequently, data of diagnostic tests for atopic manifestations (i.e. diagnostic criteria for AD, spirometry, specific IgE against food and inhalant allergens) were collected in 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 (74.5%) pediatric and 164 (79.6%) adult patients reported to have ever experienced one or more atopic manifestations compared with 28 (50.0%) partner-controls. In adult patients vs. partner-controls, prevalence of atopic dermatitis was 49.5% vs. 27.3% (p=0.003), food allergy10.7% vs. 1.9% (p=0.031), asthma 55.7% vs. 14.8% (p&lt;0.001) and allergic rhinitis 49.8% vs. 21.8% (p&lt;0.001).The frequency of current atopic manifestationsreported by patients washigher than the prevalence based on diagnostic tests (atopic dermatitis 11.2%, food allergy 1.9%, asthma 16.4% and allergic rhinitis 11.5%). Conclusions: Atopic manifestations are prevalent clinicalfeatures in a large spectrum of PIDs and in our cohort frequently present in patients with combined immune deficiencies and predominant antibody deficiencies. Evaluation of atopic manifestations should be considered in patients with PIDs.

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