Interstitial lung disease and bronchiectasis in children with primary immunodeficiencies: a single-centre experience

Abstract

Introduction: Primary immunodeficiencies (PIDs) are a heterogeneous group of inherited disorders in which one or several components of the immune system are involved. Pulmonary complications remain the most common cause of morbidity and mortality in patients with PIDs. The term chronic lung disease refers to interstitial lung disease (ILD) and bronchiectasis. Infectious as well as non-infectious factors may contribute to the development of chronic lung disease. In contrast to adults with PIDs, reports about chronic lung disease in children are limited. Prevention and early diagnosis of pulmonary complications improve patients' prognosis and quality of life. Aim of the study: To evaluate ILD and bronchiectasis in immunocompromised children. Material and methods:The study was conducted through a retrospective review of the data of 44 children with different PIDs, taking into consideration the following: pulmonary infections, autoimmunity, presence of comorbidities, and therapy performed before the diagnosis of ILD/bronchiectasis. Results: Interstitial lung disease was diagnosed in 25 cases (56.82%) and bronchiectasis in 8 cases (18.18%); both diseases were observed in 11 cases (25.00%). The earliest radiological features of ILD were detected in patients with combined PIDs. Bronchiectasis had already been reported in children above 5 years of age with a defect in antibody production. Bacterial infections requiring intravenous antibiotic treatment occurred significantly more frequently in the medical history of children with bronchiectasis (p < 0.001), whereas children with the ILD component showed a higher prevalence of pulmonary fungal infections (p = 0.034). Conclusions: Regular clinical evaluation, supported by additional tests results including radiological findings, is crucial for the early diagnosis of chronic lung disease.