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American journal of respiratory and critical care medicine
08 15, 2022;206 (4): e7-e41.

Syndrome of Combined Pulmonary Fibrosis and Emphysema: An Official ATS/ERS/JRS/ALAT Research Statement.

Vincent Cottin, Moises Selman, Yoshikazu Inoue, Alyson W Wong, Tamera J Corte, Kevin R Flaherty, MeiLan K Han, Joseph Jacob, Kerri A Johannson, Masanori Kitaichi, Joyce S Lee, Alvar Agusti, Katerina M Antoniou, Pauline Bianchi, Fabian Caro, Matias Florenzano, Liam Galvin, Tae Iwasawa, Fernando J Martinez, Rebecca L Morgan, Jeffrey L Myers, Andrew G Nicholson, Mariaelena Occhipinti, Venerino Poletti, Margaret L Salisbury, Don D Sin, Nicola Sverzellati, Thomy Tonia, Claudia Valenzuela, Christopher J Ryerson, Athol U Wells
Author Information
PMID: 35969190 DOI: 10.1164/rccm.202206-1041ST

Abstract

The presence of emphysema is relatively common in patients with fibrotic interstitial lung disease. This has been designated combined pulmonary fibrosis and emphysema (CPFE). The lack of consensus over definitions and diagnostic criteria has limited CPFE research. The objectives of this task force were to review the terminology, definition, characteristics, pathophysiology, and research priorities of CPFE and to explore whether CPFE is a syndrome. This research statement was developed by a committee including 19 pulmonologists, 5 radiologists, 3 pathologists, 2 methodologists, and 2 patient representatives. The final document was supported by a focused systematic review that identified and summarized all recent publications related to CPFE. This task force identified that patients with CPFE are predominantly male, with a history of smoking, severe dyspnea, relatively preserved airflow rates and lung volumes on spirometry, severely impaired Dl, exertional hypoxemia, frequent pulmonary hypertension, and a dismal prognosis. The committee proposes to identify CPFE as a syndrome, given the clustering of pulmonary fibrosis and emphysema, shared pathogenetic pathways, unique considerations related to disease progression, increased risk of complications (pulmonary hypertension, lung cancer, and/or mortality), and implications for clinical trial design. There are varying features of interstitial lung disease and emphysema in CPFE. The committee offers a research definition and classification criteria and proposes that studies on CPFE include a comprehensive description of radiologic and, when available, pathological patterns, including some recently described patterns such as smoking-related interstitial fibrosis. This statement delineates the syndrome of CPFE and highlights research priorities.

Keywords

diagnosis emphysema fibrosis interstitial lung disease management

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Grants

  1. 209553/Wellcome Trust
  2. 209553/Z/17/Z/Wellcome Trust
  3. /Department of Health

MeSH Term

Emphysema
Female
Humans
Hypertension, Pulmonary
Lung
Lung Diseases, Interstitial
Male
Pulmonary Emphysema
Pulmonary Fibrosis
Retrospective Studies
Syndrome
Systematic Reviews as Topic
Journal Article Research Support, Non-U.S. Gov't
Article has an altmetric score of 162

Word Cloud

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