High-resolution computed tomography and pulmonary function in children with common variable immunodeficiency

L Van Zeggeren,P.A De Jong,O.M Mets,J.M Van Montfrans,S.W.J Terheggen-Lagro,A.A.J.M Van De Ven,F.J Beek

doi:10.1183/09031936.00173410

Abstract

High-resolution computed tomography (HRCT) may be useful to monitor lung disease in children with common variable immunodeficiency disorder (CVID). We evaluated interobserver agreement and correlation with pulmonary function tests (PFTs) for automated quantification and visual scoring of air trapping and airway wall thickening on HRCT in paediatric CVID patients. In a cohort of 51 children with CVID, HRCT was analysed visually and automated for presence of air trapping and airway wall thickening. PFTs were expressed as % predicted. Disease duration, physician-diagnosed pneumonias and antibiotic prophylaxis were recorded. Interobserver agreement for automated airway wall thickening was good with an intra-class correlation coefficient of 0.88, compared with 0.51 for visual scoring. Presence of air trapping on HRCT correlated significantly with PFTs and disease duration, but was not associated with previous pneumonias. Airway wall thickening did not correlate significantly with PFTs or disease duration and was not associated with previous pneumonias or prophylactic antibiotic use. In children with CVID disorders, HRCT air trapping measurements are significantly correlated with PFTs and disease duration. Quantitative air trapping is a feasible and promising technique for small airway disease quantification that may be applied to monitor (silent) disease progression in CVID.

Highlights

We showed that in children with Common variable immunodeficiency (CVID) disorders, High-resolution computed tomography (HRCT) air trapping measurements are significantly correlated with pulmonary function tests (PFTs) and disease duration, and that interobserver agreement for HRCT airway wall thickening assessment can be improved by using automated quantitative techniques compared to visual c scores
CVID: common variable immunodeficiency; HTCT: high-resolution computed tomography; FEV1: forced expiratory volume in 1 s; % pred: % predicted; FVC: forced vital capacity; maximum midexpiratory flow at 25–75% of FVC (MMEF): maximum mid-expiratory flow at 25–75% of FVC; RV: residual volume; TLC: total lung capacity; WABSA: wall area normalised to body size; WAlung area: wall area normalised to right lung area at right upper lobe apical bronchus level; WA%age: wall area percentage corrected for age; WAPi10: wall area at a perimeter of the inner airway lumen of 10 mm; HU: Hounsfield units; ATHU-850: number of voxels, -850 HU as percentage of total number of voxels; AT15: HU at 15th percentile
We demonstrated that the presence of air trapping correlates the strongest with MMEF, a functional measure of small airways disease

Summary

Introduction

In a cohort of 51 children with CVID, HRCT was analysed visually and automated for presence of air trapping and airway wall thickening. Interobserver agreement for automated airway wall thickening was good with an intra-class correlation coefficient of 0.88, compared with 0.51 for visual scoring. Presence of air trapping on HRCT correlated significantly with PFTs and disease duration, but was not associated with previous pneumonias. Airway wall thickening did not correlate significantly with PFTs or disease. Visual more episodes of lower respiratory tract infections scores are time-consuming and associated with throughout their life [3], and structural pulmonary interobserver variation. The interobserver variahigh-resolution computed tomography (HRCT) ab- tion was substantial, especially for visual airway normalities are reported in up to 93% of patients wall thickening scores

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