D2.375 - Structural lung changes detected by chest CT in pediatric patients with combined immunodeficiencies

Children with combined immunodeficiencies (CIDs) are particularly vulnerable to chronic and recurrent respiratory infections, which may lead to early and irreversible structural lung damage.

Aim. To evaluate chest computed tomography (CT) abnormalities in pediatric patients with CIDs and to assess their association with infectious burden and timing of diagnosis.

We performed a retrospective analysis of chest CT findings in 16 children with combined immunodeficiencies from a total pediatric PID cohort of 26 patients. Radiological abnormalities were systematically reviewed and correlated with clinical history, frequency of respiratory infections, hospitalizations, and age at diagnosis.

Structural lung abnormalities were detected in 34.6% of patients (95%CI:15–54%). Atelectasis and pleural involvement were each observed in 25% of cases, while pulmonary fibrosis was identified in 12.5%. Bronchiectasis was uncommon in this relatively young cohort, likely reflecting early disease course or limited access to repeated imaging. Children with CT abnormalities had a history of recurrent pneumonias, pleural complications, and prolonged hospitalizations. Structural lung damage was more frequent in patients with delayed diagnosis and severe CID phenotypes, including severe combined immunodeficiency and DiGeorge syndrome. Delayed diagnosis (age at diagnosis >2 years) was strongly associated with structural lung disease, with affected children showing a 6.5-fold increased odds of lung damage compared to those diagnosed earlier (95% CI: 1.09–38.63).

Chest CT identifies significant early structural lung changes in children with combined immunodeficiencies. These findings underscore the critical importance of early diagnosis and aggressive infection control to prevent irreversible pulmonary damage. Chest CT should be considered an essential tool in the comprehensive evaluation and longitudinal follow-up of pediatric patients with CIDs.