![[ Visit Client Website ]](images/banner.jpg)
Pulmonary disease is a complication of many primary immunodeficiency diseases (PIDD), however few reports have compared the relative distribution of pulmonary complications across PIDDs.
METHODS:
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.
RESULTS:
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).
CONCLUSIONS:
While a common cause of morbidity, infectious and non-infectious pulmonary disease varies in type and frequency amongst PIDDs.