Table 2 Natural history of CPFE

Outcome

Study population

Comments

Worse survival

Mejia et al., 2009 [23]

CPFE vs IPF (31 vs 79)

Ominous prognosis associated with PAH (eSPAP > 75 mm Hg).

Sugino et al., 2014 [25]

CPFE vs IPF (46 vs 62)

Ominous prognosis associated with PAH (eSPAP ≥ 30.4 mm Hg). The presence of paraseptal emphysema further aggravates prognosis.

No difference

Jankowich et al., 2010 [8]

CPFE vs PF (20 vs 24)

Not restricted to IPF population.

Relatively small number of patients.

Ryerson et al., 2013 [12]

CPFE vs IPF (29 vs 336)

IPF-specific multicenter study

Large series of patients.

Usage of a prespecified threshold of ≥10 % emphysema to define CPFE.

Better survival

Kurashima et al., 2010 [37]

CPFE vs IPF, (221 vs 439)

IPF specific study.

Large series of patients.

High prevalence of CPFE (33.4 %)

Unexpectedly high median survival for the UIP population (7.5 years).

Todd et al., 2011 [38]

CPFE vs PF (54 vs 48)

Included IPF and iNSIP.

Centrilobular and not paraseptal emphysema was correlated with a worse prognosis.