Clinical characteristics associated with lung function decline in individuals with adult-diagnosed cystic fibrosis: Contemporary analysis of the Canadian CF Registry.
Individuals diagnosed with cystic fibrosis (CF) as adults represent a rare but growing subset of the CF population; there are limited studies describing their lung function trajectories.What is the overall trajectory of lung function and clinical characteristics associated with lung function decline in people diagnosed with CF as adults?and Methods: The Canadian CF Patient Registry was used to identify patients with CF who were ≥18 years of age at diagnosis and received a diagnosis between 2000 and 2017. Linear mixed effects models were used to quantify the change in lung function over age and to examine clinical characteristics associated with lung function decline.Lung function was stable in early adulthood, with a decline in middle adulthood (age 30 to 50), and a greater decline after the age of 50. Individuals diagnosed at older ages (>50 years: slope of -0.71%/year, 41 to 50: -0.68%/year, 31 to 40: -0.29%/year, 18 to 30: -0.28%/year) and those presenting with pulmonary symptoms (slope of -0.41%/year) compared with no pulmonary symptoms at baseline were associated with faster rate of lung function decline.The lung function of adult-diagnosed CF individuals in the Canadian CF registry declines slowly compared to estimates from the overall adult CF population. Adult-diagnosed individuals who are older and present with pulmonary symptoms at diagnosis experience a faster rate of lung function decline and should be monitored more closely.
Authors: Sameer Desai, Sanja Stanojevic, Grace Y Lam, Anne L Stephenson, Bradley S Quon