TY - JOUR T1 - Mortality in CPFE patients is determined by the sum of pulmonary fibrosis and emphysema JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.00316-2021 SP - 00316-2021 AU - An Zhao AU - Eyjolfur Gudmundsson AU - Nesrin Mogulkoc AU - Mark G. Jones AU - Coline van Moorsel AU - Tamera J. Corte AU - Chiara Romei AU - Recep Savas AU - Christopher J. Brereton AU - Hendrik W. van Es AU - Helen Jo-Helen AU - Annalisa De Liperi AU - Omer Unat AU - Katarina Pontoppidan AU - Frouke van Beek AU - Marcel Veltkamp AU - Peter Hopkins AU - Yuben Moodley AU - Alessandro Taliani AU - Laura Tavanti AU - Bahareh Gholipour AU - Arjun Nair AU - Sam Janes AU - Iain Stewart AU - David Barber AU - Daniel C. Alexander AU - Athol U. Wells AU - Joseph Jacob Y1 - 2021/01/01 UR - http://openres.ersjournals.com/content/early/2021/06/17/23120541.00316-2021.abstract N2 - Emphysema is one of the most common pulmonary comorbidities of idiopathic pulmonary fibrosis (IPF), presenting in about one-third of IPF patients [1]. The term combined pulmonary fibrosis and emphysema (CPFE) has been used to describe a potential phenotype characterised by the coexistence of upper lobe-predominant emphysema, lower lobe-predominant fibrosis and relative preservation of lung volumes (forced vital capacity; FVC) in the context of a disproportionately reduced gas transfer (diffusing capacity for carbon monoxide; DLCO) [1–3]. With regard to patient survival, it remains unclear whether mortality in patients with CPFE reflects the cumulative effects of two disease processes (emphysema and fibrosis), or whether CPFE represents a distinct disease phenotype where outcome is worse than the sum of disease parts (emphysema and fibrosis).FootnotesThis manuscript has recently been accepted for publication in the ERJ Open Research. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article.Conflict of interest: An Zhao has nothing to disclose.Conflict of interest: Dr. Gudmundsson has nothing to disclose.Conflict of interest: Dr. Mogulkoc has nothing to disclose.Conflict of interest: Dr. Jones has nothing to disclose.Conflict of interest: Dr. van Moorsel has nothing to disclose.Conflict of interest: Dr. Corte reports personal fees from Ad Alta, grants and personal fees from Boehringer Ingelheim, grants and personal fees from Bristol Myers Squibb, personal fees from Promedior, grants and personal fees from Roche, grants from Actelion, grants from Avalyn Pharma, grants from Biogen, grants from Galapagos, outside the submitted work; .Conflict of interest: Dr. Romei has nothing to disclose.Conflict of interest: Dr. Savas has nothing to disclose.Conflict of interest: Dr. Brereton has nothing to disclose.Conflict of interest: Dr. van Es has nothing to disclose.Conflict of interest: Dr. Jo has nothing to disclose.Conflict of interest: Dr. De Liperi has nothing to disclose.Conflict of interest: Dr. Unat has nothing to disclose.Conflict of interest: Dr. Pontoppidan has nothing to disclose.Conflict of interest: Dr. van Beek has nothing to disclose.Conflict of interest: Dr. Veltkamp has nothing to disclose.Conflict of interest: Dr. Hopkins has nothing to disclose.Conflict of interest: Dr. Moodley has nothing to disclose.Conflict of interest: Dr. Taliani has nothing to disclose.Conflict of interest: Dr. Tavanti has nothing to disclose.Conflict of interest: Dr. Gholipour has nothing to disclose.Conflict of interest: Dr. Nair reports other from Biomedical Research Centre, non-financial support from Aidence BV, Netherlands, outside the submitted work; .Conflict of interest: Dr. Janes reports personal fees and non-financial support from Astra-Zeneca, personal fees from Bard1 Bioscience, personal fees from Achilles Therapeutics, personal fees from Jansen, non-financial support from Takeda, grants from GRAIL Inc, grants from GlaxoSmithKline plc, grants from Owlstone, outside the submitted work; .Conflict of interest: Dr. Stewart has nothing to disclose.Conflict of interest: Dr. Barber has nothing to disclose.Conflict of interest: Dr. Alexander has nothing to disclose.Conflict of interest: Dr. Wells reports personal fees and non-financial support from Boehringer Ingelheim, personal fees and non-financial support from Bayer and Roche Pharmaceuticals, personal fees from Blade, outside the submitted work; .Conflict of interest: Dr. Jacob reports personal fees from Boehringer Ingelheim, personal fees from Roche, grants and personal fees from GlaxoSmithKline, personal fees from NHSX, grants from Wellcome Trust, outside the submitted work; . ER -