The prevalence of allergic manifestations in inborn errors of immunity: a retrospective cohort study

Inborn errors of immunity (IEIs) can exhibit variant clinical manifestations beyond the classic presentation of recurrent infections. Allergic diseases represent an important subset that can arise as the primary feature or coexist with IEIs. In this study, we aimed to investigate the burden of the most frequent allergic diseases among individuals with primary immune deficiency. Over 70 registered patients with a definite diagnosis of IEI were enrolled in our study. Patients and their records were evaluated, and data were collected via a questionnaire. Additionally, laboratory investigations, including spirometry, skin prick tests, and specific IgE, were used to confirm the diagnosis of allergic diseases. Statistical analysis was performed via SPSS software. The most frequent IEIs in our study comprise agammaglobulinemia, hyper-IgE syndrome, and common variable immune deficiency. The overall prevalence of allergic diseases among IEI patients was 77.8%. Allergic rhinitis, with a prevalence of 47.2%, atopic dermatitis 40.3% and asthma 37.5% were the most frequent allergic diseases among these patients. The highest prevalence of allergic rhinitis and asthma was detected among patients with agammaglobulinemia (23.5% and 33.5%). Also, Atopic dermatitis and food allergy had the highest prevalence in hyper IgE syndrome (27.6% and 75%). Agammaglobulinemia was accounted for as the most frequent IEI accompanying allergic disorders in our study. The interplay between atopy and IEIs is complex, highlighting the role of immune dysregulation in allergic diseases. Our study investigated the prevalence of allergic diseases among individuals with inborn errors of immunity. According to the coexistence of allergic diseases and IEIs, in patients with unusual, early onset, and severe allergic manifestations, underlying IEI may be suspected.