Pulmonary surveillance in inborn errors of immunity: current challenges and emerging approaches

Inborn errors of immunity comprise a heterogeneous group of disorders characterized by impaired immune function and a high burden of pulmonary complications, which are major contributors to morbidity and mortality. Lung involvement arises primarily through recurrent infection leading to airway disease, or through immune dysregulation causing non-infectious interstitial lung disease. Effective management requires sensitive tools to detect and monitor diverse pulmonary manifestations across the lifespan. Although conventional approaches, including pulmonary function testing and radiologic imaging, remain valuable, they have important limitations in this population, such as limited sensitivity, reduced feasibility in children, and cumulative radiation exposure. Emerging techniques, including multiple-breath washout and pulmonary MRI, offer radiation-free, complementary assessments of lung function and structure with the potential to improve detection and longitudinal monitoring of pulmonary complications in individuals with inborn errors of immunity.