TY - JOUR
T1 - Combined pulmonary fibrosis and emphysema as a clinicoradiologic entity
T2 - Characterization of presenting lung fibrosis and implications for survival
AU - Alsumrain, Mohammad
AU - De Giacomi, Federica
AU - Nasim, Faria
AU - Koo, Chi Wan
AU - Bartholmai, Brian J.
AU - Levin, David L.
AU - Moua, Teng
N1 - Publisher Copyright:
© 2018 Elsevier Ltd
PY - 2019/1
Y1 - 2019/1
N2 - Background: The prevalence of classifiable and unclassifiable causes of lung fibrosis and its implications for survival are mostly unknown in combined pulmonary fibrosis and emphysema (CPFE). Materials and methods: Patients with >10% involvement of both emphysema and lung fibrosis seen over 11 years at our institution were reviewed independently by expert radiologists for fibrotic and emphysematous findings and overall fibrotic CT pattern. Underlying interstitial lung disease (ILD) diagnoses and baseline demographic and clinical characteristics were collated and assessed for predictors of comparative survival. Results: In this retrospective cohort, 179 CPFE patients were identified and categorized as 58 usual interstitial pneumonia/idiopathic pulmonary fibrosis (UIP/IPF) (32%), 42 secondary ILD (23%), and 79 unclassifiable ILD (44%). The most prevalent (47%) radiologic pattern was ‘unclassifiable’ followed by ‘consistent’ and ‘possible’ UIP pattern in 38%. Adjusted predictors of mortality for the cohort as a whole included age (HR 1.03[1.01–1.06], P = 0.002), percent predicted diffusing capacity for carbon monoxide (unit HR 0.97 [0.96–0.99], P = 0.001), honeycombing (HR 1.58 [1.02–2.43], P = 0.04), and right ventricular dysfunction (HR 2.28 [1.39–3.97], P = 0.002). Survival was similar between CPFE with secondary ILD and CPFE with UIP/IPF, while CPFE with unclassifiable ILD had better comparative survival (Log rank = 0.026). Conclusions: Our findings suggest only about a third of CPFE patients represent suspected UIP/IPF; the majority were clinically and radiologically unclassifiable ILD whose survival was comparatively better. Identifiable or secondary causes of lung fibrosis in CPFE occurred in about a fifth of presenting patients.
AB - Background: The prevalence of classifiable and unclassifiable causes of lung fibrosis and its implications for survival are mostly unknown in combined pulmonary fibrosis and emphysema (CPFE). Materials and methods: Patients with >10% involvement of both emphysema and lung fibrosis seen over 11 years at our institution were reviewed independently by expert radiologists for fibrotic and emphysematous findings and overall fibrotic CT pattern. Underlying interstitial lung disease (ILD) diagnoses and baseline demographic and clinical characteristics were collated and assessed for predictors of comparative survival. Results: In this retrospective cohort, 179 CPFE patients were identified and categorized as 58 usual interstitial pneumonia/idiopathic pulmonary fibrosis (UIP/IPF) (32%), 42 secondary ILD (23%), and 79 unclassifiable ILD (44%). The most prevalent (47%) radiologic pattern was ‘unclassifiable’ followed by ‘consistent’ and ‘possible’ UIP pattern in 38%. Adjusted predictors of mortality for the cohort as a whole included age (HR 1.03[1.01–1.06], P = 0.002), percent predicted diffusing capacity for carbon monoxide (unit HR 0.97 [0.96–0.99], P = 0.001), honeycombing (HR 1.58 [1.02–2.43], P = 0.04), and right ventricular dysfunction (HR 2.28 [1.39–3.97], P = 0.002). Survival was similar between CPFE with secondary ILD and CPFE with UIP/IPF, while CPFE with unclassifiable ILD had better comparative survival (Log rank = 0.026). Conclusions: Our findings suggest only about a third of CPFE patients represent suspected UIP/IPF; the majority were clinically and radiologically unclassifiable ILD whose survival was comparatively better. Identifiable or secondary causes of lung fibrosis in CPFE occurred in about a fifth of presenting patients.
KW - CPFE
KW - Combined pulmonary fibrosis and emphysema
KW - Survival
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U2 - 10.1016/j.rmed.2018.12.003
DO - 10.1016/j.rmed.2018.12.003
M3 - Article
C2 - 30665507
AN - SCOPUS:85058802823
SN - 0954-6111
VL - 146
SP - 106
EP - 112
JO - Respiratory Medicine
JF - Respiratory Medicine
ER -