A systematic review of studies examining the rate of lung function decline in patients with cystic fibrosis.
A systematic review was performed (i) to describe the reported overall rate of progression of CF lung disease quantified as FEV1%predicted decline with age, (ii) to summarise identified influencing risk factors and (iii) to review methods used to analyse CF lung disease progression data. A search of publications providing FEV1%predicted values over age was conducted in PUBMED and EMBASE. Baseline and rate of FEV1%predicted decline were summarised overall and by identified risk factors. Thirty-nine studies were included and reported variable linear rates of lung function decline in patients with CF. The overall weighted mean FEV1%predicted over age was graphically summarised and showed a nonlinear, time-variant decline of lung function. Compared to their peers, Pseudomonas aeruginosa infection and pancreatic insufficiency were most commonly associated with lower baseline and more rapid FEV1%predicted declines respectively. Considering nonlinear models and drop-out in lung disease progression, analysis is lacking and more studies are warranted.
School of Pharmacy, The University of Queensland, Brisbane QLD 4072, Australia, School of Pharmaceutical Sciences, Universiti Sains Malaysia, 11800 USM, Pulau Pinang, Malaysia. Electronic address: s.harun@uq.edu.au.
Sabariah Noor
SN
Harun
Department of Respiratory and Sleep Medicine Lady Cilento Children's Hospital South Brisbane, Queensland 4101, Queensland Children's Medical Research Institute, Herston Rd, Herston QLD, 4029, and School of Medicine, The University of Queensland Brisbane, QLD 4072, Australia. Electronic address: Claire.Wainwright@health.qld.gov.au.
Claire
C
Wainwright
Statistics/Clinical Trials and Biostatistics Unit, QIMR Berghofer Medical Research Institute, Royal Brisbane Hospital QLD 4029 Australia. Electronic address: Kerenaftali.Klein@qimrberghofer.edu.au.
Kerenaftali
K
Klein
School of Pharmacy, The University of Queensland, Brisbane QLD 4072, Australia. Electronic address: s.hennig@uq.edu.au.
Stefanie
S
Hennig
D002648:Child; D003550:Cystic Fibrosis / Q000503:physiopathology*; D018450:Disease Progression; D005541:Forced Expiratory Volume / Q000502:physiology*; D006801:Humans; D008168:Lung / Q000503:physiopathology*; D012129:Respiratory Function Tests; D012307:Risk Factors
Cystic fibrosis; disease progression; lung function; nonlinear decline; risk factors