Non-CF bronchiectasis in pediatrics: a cohort profile of patients with inborn errors of immunity at a referral center in Cali

Introduction. Inborn errors of immunity (IEI) are frequently associated with bronchiectasis. The diagnostic performance of IEI has improved because the association of some of these entities with progressive airway damage is better known with certainty. This has allowed recognition and appropriate intervention, reducing lung function and quality of life. Objective. . To describe a group of patients with bronchiectasis not related to cystic fibrosis (CF) who were diagnosed with IEI and studied in an immunology reference center in Colombia. Materials and methods. Observational, descriptive, and retrospective study examined patients under 18 diagnosed with IEI and non-CF bronchiectasis between December 2013 and December 2023 at a referral center in Colombia (Fundaci & oacute;n Valle del Lili, Cali). Results. A total of 17 patients were diagnosed with both non-CF bronchiectasis and IEI. The mean age was 9 years. The lower lobe was the most frequently affected segment, and in most cases, the distribution was unilateral. The most prevalent alteration was predominant antibody deficiency, followed by combined immunodeficiencies associated with syndromes. It was observed that 76% of cases demonstrated involvement of humoral immunity, while 23% exhibited alterations of both humoral and cellular immunity. Additionally, 70% of patients exhibited genetic alterations related to their phenotype. Of these patients, 76% underwent supplementation with intravenous immunoglobulin (Ig IV), and 18% died. Conclusion. The IEIs most frequently associated with non-CF bronchiectasis were predominant antibody deficiency and combined immunodeficiencies with syndromic features.