Primary immunodeficiencies due to IgA deficiency. Clinic heterogeneity and diagnostic challenge
Keywords:
SELECTIVE IGA DEFICIENCY, PRIMARY IMMUNODEFICIENCY DISEASES, INBORN ERROR OF IMMUNITY, IGA DEFICIENCY.Abstract
Introduction: selective immunoglobulin A (IgA) deficiency is the most common primary immunodeficiency (PID); it can go unnoticed causing false diagnoses and unexplained reactions to blood products. Data on the main characteristics of patients with PID due to IgA deficiency (PID-IgA) are relatively scarce; hence, this knowledge is crucial for a better identification and management of this disease.
Objective: to characterize patients with primary immunodeficiency due to IgA deficit in patients diagnosed with PID-IgA, assisted in the Immunology and Allergy service of "Carlos Manuel de Céspedes" Provincial University Hospital, in Bayamo-Granma, from 2013 to 2022.
Methods: a descriptive, retrospective study was conducted in the total of patients (22) diagnosed with PID-IgA (partial and total). Demographic variables were studied, as well as other such as clinical features, familial antecedents and type of IgA deficiency.
Results: mean age of symptoms onset was three years. 54,5 % of patients diagnosed with PID-IgA were female and 68,2 % suffered from a partial deficit. Allergies, infections and autoimmunity presented in 77,2 %, 68,2 % and 31,8 % of total cases, respectively. Family history of allergies was reported in 59,1 % of patients and familial PID in 27,3 %.
Conclusions: The main features that characterized PID-IgA in Granma were a greater incidence in females, predominance of the partial deficiency type, clinical onset in childhood for symptomatic patients and the report of allergic diseases as the most frequently reported clinical feature and familial antecedent.
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