Combined pulmonary fibrosis and emphysema as a clinicoradiologic entity: Characterization of presenting lung fibrosis and implications for survival

Mohammad Alsumrain, Federica De Giacomi, Faria Nasim, Chi Wan Koo, Brian Jack Bartholmai, David L. Levin, Teng Moua

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Pages (from-to)106-112
Number of pages7
JournalRespiratory Medicine
Volume146
DOIs
StatePublished - Jan 1 2019

Fingerprint

Pulmonary Emphysema
Pulmonary Fibrosis
Idiopathic Pulmonary Fibrosis
Interstitial Lung Diseases
Fibrosis
Lung
Survival
Right Ventricular Dysfunction
Emphysema
Carbon Monoxide
Demography
Mortality

Keywords

  • Combined pulmonary fibrosis and emphysema
  • CPFE
  • Survival

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Combined pulmonary fibrosis and emphysema as a clinicoradiologic entity : Characterization of presenting lung fibrosis and implications for survival. / Alsumrain, Mohammad; De Giacomi, Federica; Nasim, Faria; Koo, Chi Wan; Bartholmai, Brian Jack; Levin, David L.; Moua, Teng.

In: Respiratory Medicine, Vol. 146, 01.01.2019, p. 106-112.

Research output: Contribution to journalArticle

Alsumrain, Mohammad ; De Giacomi, Federica ; Nasim, Faria ; Koo, Chi Wan ; Bartholmai, Brian Jack ; Levin, David L. ; Moua, Teng. / Combined pulmonary fibrosis and emphysema as a clinicoradiologic entity : Characterization of presenting lung fibrosis and implications for survival. In: Respiratory Medicine. 2019 ; Vol. 146. pp. 106-112.
@article{ebd3b5dbe2244615a560f1a6ec51e704,
title = "Combined pulmonary fibrosis and emphysema as a clinicoradiologic entity: Characterization of presenting lung fibrosis and implications for survival",
abstract = "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.",
keywords = "Combined pulmonary fibrosis and emphysema, CPFE, Survival",
author = "Mohammad Alsumrain and {De Giacomi}, Federica and Faria Nasim and Koo, {Chi Wan} and Bartholmai, {Brian Jack} and Levin, {David L.} and Teng Moua",
year = "2019",
month = "1",
day = "1",
doi = "10.1016/j.rmed.2018.12.003",
language = "English (US)",
volume = "146",
pages = "106--112",
journal = "Respiratory Medicine",
issn = "0954-6111",
publisher = "W.B. Saunders Ltd",

}

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 Jack

AU - Levin, David L.

AU - Moua, Teng

PY - 2019/1/1

Y1 - 2019/1/1

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 - Combined pulmonary fibrosis and emphysema

KW - CPFE

KW - Survival

UR - http://www.scopus.com/inward/record.url?scp=85058802823&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85058802823&partnerID=8YFLogxK

U2 - 10.1016/j.rmed.2018.12.003

DO - 10.1016/j.rmed.2018.12.003

M3 - Article

VL - 146

SP - 106

EP - 112

JO - Respiratory Medicine

JF - Respiratory Medicine

SN - 0954-6111

ER -