Pulmonary disease is a complication of many primary immunodeficiency diseases (PIDD), however few reports have compared the relative distribution of pulmonary complications across PIDDs.
We analyzed 3610 subjects in the USIDNET registry. Descriptive and nonparametric statistics were utilized to characterize the types and frequency of infectious and non-infectious pulmonary conditions across PIDD categories.
Pneumonia was the most frequent infection type overall (42.3%) with highest rates in phagocyte defects (54.0%) and lowest rates in complement disorders (12.0%). Bronchitis (17.9%), abscesses (3%), bronchiolitis (2.4%), and empyema (0.5%) were less common. The overall majority of pulmonary infections were bacterial (49.5%), although fungal organisms were predominant in phagocytic (52.8%) and innate immune (52.9%) defects. Non-infectious pulmonary disease (30.8% overall) included asthma/reactive airway disease (21.7%), bronchiectasis (7.0%), and interstitial lung disease (4.2%). Subjects with non-infectious lung disease were more likely to be >18 years and have had a history of pulmonary infection compared to those without non-infectious lung disease (p<0.00001). Most subjects (69.7%) received IVIG with highest treatment rates in antibody deficiencies (89.3%) and lowest in phagocytic defects (27.9%). Fewer subjects received prophylactic (11%) and treatment (13.8%) antibiotics. More subjects with infectious conditions than without were treated with IVIG (p<0.00001). Antibiotic prophylaxis was more common in subjects with non-infectious pulmonary disease versus those without disease (p<0.00001). Subjects with earlier initiation of IVIG treatment were less likely to report infectious and non-infectious pulmonary complications (p<0.00001).
While a common cause of morbidity, infectious and non-infectious pulmonary disease varies in type and frequency amongst PIDDs.