Bronchiectasis Secondary to Primary Immunodeficiency in Children: Longitudinal Changes in Structure and Function

Background: Primary immunodeficiency is a common cause of bronchiectasis in children. The term bronchiectasis suggests an irreversible process; however, disease progression following treatment is controversial. The aim of this study was to evaluate the progression of bronchiectasis in children with primary immunodeficiency after institution of treatment. Methods: A retrospective review of case notes of children with primary immunodeficiency was undertaken to identify patients with confirmed bronchiectasis. Children who had two high-resolution computed tomography scans of the chest (HRCT chest) with an interval of at least 2 years were identified. The HRCT-chest scans at diagnosis and follow up were scored using a Bhalla score. Spirometry results (FEV1, FVC, and FEV1:FVC ratios) were related to HRCT-chest scores, where available. Statistical analysis was by Wilcoxon signed rank test and Spearman's rank order correlation. Results: Eighteen subjects were studied. The diagnosis of primary immunodeficiency was established at median (range) age 3.4 (1-13) years, and bronchiectasis at 9.3 (3.1-13.8) years. There was no significant difference between baseline and follow-up median (range) HRCT-chest scores (6 [1-13] and 7.5 (0-15], P = 0.21) respectively. The follow-up FEV1 and FVC percent predicted median (range) were significantly higher than baseline (86% [49-124%] vs. 75% [36-93%], P<0.005, and 86% [47-112%] vs. 78% [31-96%], P<0.05), respectively; there was no significant difference between baseline and follow-up FEV1:FVC ratios. There was no significant correlation between HRCT-chest score changes and FEV(1)or FVC changes. Conclusions: Bronchiectasis secondary to primary immunodeficiency in childhood is not always a progressive condition, suggesting a potential to slow or prevent disease progression with appropriate treatment. Pediatr Pulmonol. 2009; 44:669-675. (C) 2009 Wiley-Liss, Inc.