Background:Combined pulmonary fibrosis and emphysema (CPFE) is a syndrome characterized
by the coexistence of upper lobe emphysema and lower lobe fibrosis. However,
whether CPFE has a higher or lower mortality than idiopathic pulmonary
fibrosis (IPF) alone is still not clear. In this study we conducted a
meta-analysis to assess the survival rate (SR) of CPFE
versus IPF alone in clinical trials.Methods:We performed a systematic search of PubMed, Embase, and the Cochrane Central
Register of Controlled Trials for trials published prior to 31 March 2018.
Extracts from the literature were analyzed with Review Manager version
5.3.Results:Thirteen eligible trials were included in this analysis (involving 1710
participants). Overall, the pooled results revealed that no statistically
significant difference was detected in the 1-year [relative risk (RR) =
0.98, 95% confidence interval (CI): 0.94–1.03, p = 0.47],
3-year (RR = 0.83, 95% CI: 0.68–1.01, p = 0.06), and 5-year
(RR = 0.80, 95% CI: 0.59–1.07, p = 0.14) SRs of CPFE
versus IPF alone.Conclusions:CPFE exhibits a very poor prognosis, similar to IPF alone. Additional studies
are needed to provide more convincing data to investigate the natural
history and outcome of patients with CPFE in comparison to IPF.
The reviews of this paper are available via the supplemental
material section.