A CT scan score for the assessment of lung disease in children with common variable immunodeficiency disorders.

Abstract

The prevalence and severity of structural lung disease in children with common variable immunodeficiency (CVID) disorders is not well known, and a dedicated CT scanning protocol and CT scan scoring system have not been described in this category. This was a cohort study of 54 children (34 CVID, 20 CVID-like disorder) in a stable condition who underwent volumetric inspiratory and end-expiratory CT scans. Scans were scored for airway abnormalities, interstitial and parenchymal lung disease, and lymphadenopathy using a newly developed CT scan scoring system. Scores were normalized to a 0% to 100% scale. Observer agreement was assessed using an intraclass correlation coefficient (Ri). Prevalence and severity of CT scan abnormalities were calculated. Structural lung disease was common (85%-93%), but usually mild as reflected in the relatively low scores (bronchiectasis score 2.8% +/- 6.4%). Moderate-to-severe bronchiectasis was found in three (5%) patients. Expiratory air trapping was the most common finding, found in 71% to 80%, but often in a mild form; application of a cut off level of > 10% reduced its prevalence to 33% to 38%. In 9% to 15% of all patients, air trapping was the only abnormality. Multiple lung nodules were seen in 24% to 25% and could disappear after corticosteroid treatment. Observer agreement was moderate (Ri 0.6-0.79) to good (Ri > 0.8) for all items and the composite scores, except airway wall thickening. In children with CVID disorders, mild structural lung disease is common. Expiratory CT scans show the most frequent abnormality, air trapping. The occurrence of (silent) lung disease progression and the clinical impact of CT scans require further investigations.