PURPOSE:Pulmonary diseases contribute significantly to the morbidity in primary immunodeficiency (PID) but their incidence and prevalence is unknown.
METHODS:We undertook a historical cohort study in Olmsted County, MN, USA from January 1, 1976 to December 31, 2006 of subjects with a diagnosis of PID and evaluated the prevalence and spectrum of pulmonary diseases. The charts of all patients with PID were abstracted for laboratory parameters and 4 specific clinical diagnoses: bronchiectasis, asthma, chronic obstructive pulmonary disease (COPD) and interstitial lung disease. The diagnosis of pulmonary disease was verified by clinical and radiological documentation. The data were analyzed using the Wilcoxon signed rank test and univariate and multivariate regression models.
RESULTS:There were 172 cases of PID diagnosed in the 31-year time period. Seventy-three (43%) of these patients had one or more pulmonary condition, including 53 (31%) with bronchiectasis, 11 (6%) with COPD, 6 (4%) with asthma, and 3 (2%) with interstitial lung disease. Lower serum immunoglobulin levels were correlated with the presence of lung diseases but only lower IgG2 subclass levels were correlated with the presence of bronchiectasis (P=0.008). Bronchiectasis was a significant predictor of prophylactic antibiotic usage (odds ratio 2.4; 95% CI=1.02, 6.06;P=0.04) and was also associated with mortality (odds ratio 3.6; 95% CI= 0.98, 13.4; P=0.05).
CONCLUSION:Pulmonary complications, particularly bronchiectasis, are commonly associated with PID. The presence of IgG2 deficiency correlates with bronchiectasis in these patients.
CLINICAL IMPLICATIONS:Pulmonary diseases contributed substantially to the overall disease burden in our cohort of subjects with PID. Unexplained bronchiectasis should prompt an evaluation for an underlying immunodeficiency disorder.
DISCLOSURE:Avni Joshi, No Financial Disclosure Information; No Product/Research Disclosure Information