Pulmonary Abnormalities on High-Resolution CT Demonstrate More Rapid Decline Than FEV1 in Adults With Cystic Fibrosis

Pulmonary Abnormalities on High-Resolution CT Demonstrate More Rapid Decline Than FEV1 in Adults With Cystic FibrosisCystic fibrosis (CF) is the most common autosomal recessive inherited disorder in whites, with a prevalence of 1 in 1,461 births in the Irish population. By the later teenage years, significant irreversible lung damage has occurred in many patients. The majority die of recurrent respiratory sepsis and respiratory failure in adulthood.
Spirometry, particularly FEV1, is used as a marker of lung disease because of its reproducibility and accuracy as an outcome surrogate. It is well known that spirometry results decline as lung disease progresses. In a thought-provoking article, de Jong et al showed that the rate of decline in high-resolution CT (HRCT) appearances was more significant than the rate of decline in spirometry results in children, and this has been confirmed by others. More recently, further novel work has shown that several HRCT abnormalities decline at a faster rate than FEV1 in adults. As a result, proposals for the use of HRCT as an outcome surrogate have been suggested.” In this regard, a combined HRCT/ spirometric score has been shown to be a sensitive marker in detecting treatment effects. More recently, HRCT has been shown to correlate with the number of respiratory exacerbations in children over time.
In adults with CF, the rate of change in spirometry is well documented, but the rate of change in HRCT abnormalities has only been described in a single study. Since adults generally have more progressive lung function impairment than children, we hypothesized that the rate of decline in lung spirometry in this age group would be similar or worse than the rate of decline in HRCT abnormalities. The aim of this study was to compare the rates of change in HRCT and clinical measurements over a significant time period in an adult cohort of patients with CF.
Since 1997, all adult patients undergo elective HRCT every 18 to 24 months as part of scheduled long-term patient assessment at the Irish National Referral Centre for Adult CF. Patients > 16 years old from the entire country are referred to the center for multidisciplinary care. The number of patients attending our center has steadily increased from 1997 (approximately 170 patients) to 2005 (approximately 250 patients) as treatment has become more centralized and life expectancy continues to improve.

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