Progression of lung disease in primary ciliary dyskinesia: Is spirometry less accurate than CT?

• Published in: Pediatric pulmonology Vol. 47; no. 5; pp. 498 - 504
• Main Authors: Maglione, Marco, Bush, Andrew, Montella, Silvia, Mollica, Carmine, Manna, Angelo, Esposito, Antonietta, Santamaria, Francesca
• Format: Journal Article
• Language: English
• Published: Hoboken Wiley Subscription Services, Inc., A Wiley Company 01-05-2012; Wiley-Liss
• Subjects: Adolescent; Adult; Biological and medical sciences; Bronchiectasis - diagnostic imaging; Bronchiectasis - physiopathology; Child; Complex syndromes; Disease Progression; Female; Forced Expiratory Volume - physiology; General aspects; high-resolution computed tomography; Humans; Kartagener Syndrome - diagnostic imaging; Kartagener Syndrome - physiopathology; lung disease; Male; Medical genetics; Medical sciences; Pneumology; primary ciliary dyskinesia; Retrospective Studies; Severity of Illness Index; Spirometry; Tomography, X-Ray Computed; Young Adult; Lung disease; Pediatrics; Spirometry; High resolution; Radiodiagnosis; Respiratory disease; high-resolution computed tomography; Situs inversus; Immotile cilia syndrome; Exploration; Congenital disease; Lung function; Malformation; Medical imagery; ENT disease; Computerized axial tomography; primary ciliary dyskinesia; Pneumology
• ISSN: 8755-6863; 1099-0496
• DOI: 10.1002/ppul.21569

Despite its extensive use, there is no evidence that spirometry is useful in the assessment of progression of lung disease in primary ciliary dyskinesia (PCD). We hypothesize that high‐resolution computed tomography (HRCT) is a better indicator of PCD lung disease progression than spirometry. We retrospectively evaluated two paired spirometry and HRCT examinations from 20 PCD patients (age, 11.6 years; range, 6.5–27.5 years). The evaluations were performed in stable state and during unstable lung disease. HRCT scans were scored blind by two raters. Compared to the first assessment, at the second evaluation spirometry did not change while HRCT scores significantly worsened (P < 0.01). Age was significantly related to HRCT total (r = 0.5; P = 0.02) and bronchiectasis scores (r = 0.5; P = 0.02). At both evaluations, HRCT total score correlated with FEV1 (r = −0.5, P = 0.01; r = −0.7, P = 0.001, respectively) and FVC Z scores (r = −0.6, P = 0.006; r = −0.7, P = 0.001, respectively), and bronchiectasis score was related to FEV1 (r = −0.5, P = 0.03; r = −0.6; P = 0.002, respectively) and FVC Z scores (r = −0.6, P = 0.008; r = −0.7, P = 0.001, respectively). No relationship was found between the change in HRCT scores and the change in spirometry. In PCD, structural lung disease may worsen despite spirometry being stable. Pediatr Pulmonol. 2012; 47:498–504. © 2011 Wiley Periodicals, Inc.