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

Cottin, Vincent; Selman, Moises; Inoue, Yoshikazu; Wong, Alyson W; Corte, Tamera J; Flaherty, Kevin R; Han, MeiLan K; Jacob, Joseph; Johannson, Kerri A; Kitaichi, Masanori; Lee, Joyce S; Agusti, Alvar; Antoniou, Katerina M; Bianchi, Pauline; Caro, Fabian; Florenzano, Matias; Galvin, Liam; Iwasawa, Tae; Martinez, Fernando J; Morgan, Rebecca L; ... (2022). Syndrome of Combined Pulmonary Fibrosis and Emphysema: An Official ATS/ERS/JRS/ALAT Research Statement. American journal of respiratory and critical care medicine, 206(4), e7-e41. American Thoracic Society 10.1164/rccm.202206-1041ST

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Background: 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. Goals: 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. Methods: 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. Results: 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 DlCO, 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. Conclusions: This statement delineates the syndrome of CPFE and highlights research priorities.

Item Type:

Journal Article (Review Article)

Division/Institute:

04 Faculty of Medicine > Pre-clinic Human Medicine > Institute of Social and Preventive Medicine (ISPM)

UniBE Contributor:

Tonia, Thomai

Subjects:

600 Technology > 610 Medicine & health
300 Social sciences, sociology & anthropology > 360 Social problems & social services

ISSN:

1535-4970

Publisher:

American Thoracic Society

Language:

English

Submitter:

Doris Kopp Heim

Date Deposited:

19 Jan 2023 12:30

Last Modified:

20 Mar 2023 14:21

Publisher DOI:

10.1164/rccm.202206-1041ST

PubMed ID:

35969190

Uncontrolled Keywords:

diagnosis emphysema fibrosis interstitial lung disease management

BORIS DOI:

10.48350/177685

URI:

https://boris.unibe.ch/id/eprint/177685

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