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Table 2 Natural history of CPFE

From: Combined Pulmonary Fibrosis and Emphysema, a clinical review

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.