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Allergic comorbidities among inborn errors of immunity in children attended in a high-complexity center in Cali,
Jacobo Triviño-Arias1, Sofia Martínez-Betancur1, Oriana Arias-Valderrama1,2
1Fundación Valle del Lili, Centro de Investigaciones Clínicas, Cali, Colombia.
Insights
Allergic diseases (AD) are common in children with inborn errors of immunity (IEI), often distinct from primary atopic diseases. Recognizing these comorbidities aids early IEI diagnosis and management.
Area of Science:
- Pediatric Immunology
- Allergy and Immunology
- Genetics
Background:
- Inborn errors of immunity (IEI) are a diverse group of genetic disorders affecting the immune system.
- Allergic diseases (AD) are frequently observed in patients with IEI, but their distinct clinical characteristics as comorbidities require further elucidation.
- Differentiating AD as comorbidities from primary atopic diseases (PAD) is crucial for accurate diagnosis and management.
Purpose of the Study:
- To describe the clinical characteristics of allergic diseases (AD) as comorbidities in pediatric patients diagnosed with inborn errors of immunity (IEI).
- To distinguish these allergic comorbidities from primary atopic diseases (PAD).
Main Methods:
- An observational, retrospective study included pediatric patients diagnosed with IEI and allergic comorbidity between 2013-2023.
- Diagnoses were made according to ESID and IUIS-2022 criteria.
- Descriptive analysis of variables and odds ratio (OR) for allergic sensitization were performed using R Studio.
Main Results:
- Out of 366 IEI patients, 238 (65%) had concomitant AD.
- The most common IEI types were antibody-specific deficiencies (84%) and combined immunodeficiencies (2.5%).
- The most frequent ADs were asthma (48.7%), rhinitis (18.9%), and atopic dermatitis (15.1%).
Conclusions:
- Allergic diseases are increasingly recognized as indicators for early IEI diagnosis.
- AD can coexist with IEI without being primary atopic diseases, necessitating comprehensive care.
- Recognizing this heterogeneity supports a multidisciplinary approach for improved early detection and management of IEI.
Objective:
To describe the clinical characteristics of allergic diseases (AD) as comorbidities-distinct from primary atopic diseases (PAD)-in pediatric patients diagnosed with IEI.
Methods:
An observational and retrospective study was made. We included pediatric population diagnosed with IEI and allergic comorbidity between 2013-2023. The diagnosis was done considering the criteria from the ESID and IUIS-2022. We performed a descriptive analysis of the variables (frequencies/percentages). For qualitative variables, we used OR to obtain the probability of occurrence of allergic sensitization among patients with AD and IEI. Every analysis was performed with the software R Studio version 3.2.2.
Results:
There were 366 patients with diagnosis of IEI, 238 had concomitant AD. 59.6% were males. Mean age was 5 years. 84% had antibody-specific deficiencies, 8% combined immunodeficiencies associated to well-defined syndromes, 2.9% congenital defects in phagocytes, 2.5% combined-immunodeficiencies, 1.7% autoinflammatory diseases, and 0.8% defects in intrinsic and innate immunity. The distribution of the ADs was asthma 48.7%, rhinitis 18.9%, atopic-dermatitis 15.1%, food-allergy 8.4%, acute urticaria 5%, and chronic urticaria 3.8%.
Conclusion:
Allergic diseases are increasingly recognized as key indicators for early IEI diagnosis. These conditions may coexist without representing PAD, emphasizing the need for comprehensive care. Recognizing this heterogeneity supports a multidisciplinary approach to improve early detection and management of IEI.
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