Please use this identifier to cite or link to this item: http://ahro.austin.org.au/austinjspui/handle/1/23040
Title: Pleuroparenchymal fibroelastosis in systemic sclerosis: prevalence and prognostic impact.
Authors: Bonifazi, Martina;Sverzellati, Nicola;Negri, Eva;Jacob, Joseph;Egashira, Ryoko;Moser, Joanna;Piciucchi, Sara;Mei, Federico;De Lauretis, Angelo;Visca, Dina;Goh, Nicole S L;Bonini, Matteo;Cirilli, Laura;La Vecchia, Carlo;Chua, Felix;Kouranos, Vasileios;Margaritopoulos, George;Kokosi, Maria;Maher, Toby M;Gasparini, Stefano;Gabrielli, Armando;Wells, Athol U;Renzoni, Elisabetta A
Affiliation: Department of Cardiovascular and Thoracic Sciences, Fondazione Policlinico Universitario A.Gemelli- IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy
National Heart and Lung Institute (NHLI), Imperial College London and Royal Brompton Hospital, London
Interstitial Lung Disease Unit, Royal Brompton Hospital, Imperial College, London, UK
Department of Biomedical Sciences and Public Health, Università Politecnica delle Marche, Ancona, Italy. Pulmonary Disease Unit, Department of Internal Medicine, Azienda Ospedali Riuniti, Ancona, Italy
Pulmonary Diseases Unit, Azienda Ospedaliera "Guido Salvini", Garbagnate Milanese, Italy
Division of Pulmonary Rehabilitation, Istituti Clinic Scientifici Maugeri, IRCCS, Tradate, Italy
Interstitial Lung Disease Unit, Manchester University Hospital NHS FT, Wythenshawe Hospital, Manchester, UK
Interstitial Lung Disease Unit, Royal Brompton Hospital, Imperial College, London, UK
Pulmonary Disease Unit, Department of Internal Medicine, Azienda Ospedali Riuniti, Ancona, Italy
Radiology, Department of Medicine and Surgery, Università di Parma, Parma, Italy
Department of Respiratory Medicine, University College London, London, UK
Centre for Medical Image Computing, University College London, London, UK
Department of Respiratory and Sleep Medicine, Austin Health, Heidelberg, Victoria, Australia
Institute for Breathing and Sleep, Austin Health, Heidelberg, Victoria, Australia
Department of Biomedical Sciences and Public Health, Università Politecnica delle Marche, Ancona, Italy
Department of Biomedical and Clinical Sciences "Luigi Sacco", Università degli Studi di Milano, Milano, Italy
Department of Radiology, Faculty of Medicine, Saga University, Saga city, Japan
Department of Radiology, St George's University Hospitals NHS Foundation Trust, London, UK
Radiology Unit, Ospedale GB Morgagni, Forlì, Italy
Pulmonary Disease Unit, Department of Internal Medicine, Azienda Ospedali Riuniti, Ancona, Italy
Department of Biomedical Sciences and Public Health, Università Politecnica delle Marche, Ancona, Italy
Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy
Interstitial Lung Disease Unit, Royal Brompton Hospital, Imperial College, London, UK
Department of Clinical and Molecular Sciences, Università Politecnica delle Marche, Ancona, Italy
Interstitial Lung Disease Unit, Royal Brompton Hospital, Imperial College, London, UK
Issue Date: 16-Apr-2020
EDate: 2020-04-16
Citation: The European respiratory journal 2020; online first: 16 April
Abstract: Interstitial lung disease (ILD) in systemic sclerosis (SSc) is a major cause of morbidity and mortality, mostly presenting as nonspecific interstitial pneumonia. Little is known about the prevalence of pleuroparenchymal fibroelastosis (PPFE), a specific entity affecting the visceral pleura and subpleural parenchyma. We set out to estimate PPFE prevalence in two large cohorts of SSc patients and to assess its impact on survival and functional decline.A total of 359 SSc patients, derived from two referral centers in two different countries (UK and Italy), were included. The first available high-resolution computed tomography scan was independently evaluated by two radiologists blind to clinical information, to quantify ILD extent, freestanding bronchial abnormalities, and lobar percentage involvement of PPFE on a 4-point categorical scale. Discordant scores were adjudicated by a third scorer. PPFE extent was further classified as limited (≤2/18) or extensive (>2/18). Results were evaluated against functional decline and mortality.The overall prevalence of PPFE in the combined SSc population was 18% (11% with extensive PPFE), with no substantial difference between the two cohorts. PPFE was significantly linked to free-standing bronchial abnormalities (61% versus 25% in PPFE versus no PPFE; p<0.0001) and to worse survival, independently of ILD severity or short-term lung function changes (HR 1.89, 95% CI 1.10-3.25; p=0.005).In the current study, we provide an exhaustive description of PPFE prevalence and clinical impact in the largest cohort of SSc subjects published so far. PPFE presence should be carefully considered, due to its significant prognostic implications.
URI: http://ahro.austin.org.au/austinjspui/handle/1/23040
DOI: 10.1183/13993003.02135-2019
ORCID: 0000-0002-8054-2293
0000-0003-2065-4346
PubMed URL: 32299855
Type: Journal Article
Appears in Collections:Journal articles

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