Risk factors for rate of decline in FEV1 in adults with cystic fibrosis

J Cyst Fibros. 2012 Sep;11(5):405-11. doi: 10.1016/j.jcf.2012.03.009. Epub 2012 May 5.


Background: Previously we assessed risk factors for FEV(1) decline in children and adolescents using the Epidemiologic Study of Cystic Fibrosis (J Pediatr 2007;151:134-139); the current study assessed risk factors in adults.

Methods: Risk factors for FEV(1) decline over 3-5.5 years for ages 18-24 and ≥25 years were assessed using mixed-model regression.

Results: Mean rates of FEV(1) decline (% predicted/year) were -1.92 for ages 18-24y (n=2793) and -1.45 for ages ≥25y (n=1368). For the 18-24y group, B. cepacia, pancreatic enzyme use, multidrug-resistant P. aeruginosa, cough, mucoid P. aeruginosa, and female sex predicted greater decline; low baseline FEV(1) and sinusitis predicted less decline. For the ≥25y group, only pancreatic enzyme use predicted greater decline; low baseline FEV(1) and sinusitis predicted less decline.

Conclusions: Risk factors for FEV(1) decline in adults <25 years are similar to those previously identified in children and adolescents; older adults had few statistically significant risk factors.

MeSH terms

  • Adult
  • Age Factors
  • Cystic Fibrosis / complications
  • Cystic Fibrosis / diagnosis
  • Cystic Fibrosis / epidemiology*
  • Cystic Fibrosis / physiopathology*
  • Cystic Fibrosis / therapy
  • Disease Progression
  • Enzyme Replacement Therapy / statistics & numerical data
  • Female
  • Humans
  • Male
  • Nutritional Status
  • Pancreas / enzymology
  • Pseudomonas Infections / physiopathology*
  • Pseudomonas aeruginosa / isolation & purification*
  • Regression Analysis
  • Respiratory Function Tests* / methods
  • Respiratory Function Tests* / statistics & numerical data
  • Respiratory System / physiopathology*
  • Respiratory Tract Infections / microbiology
  • Respiratory Tract Infections / physiopathology
  • Risk Factors
  • Sex Factors