TY - JOUR
T1 - Pleuroparenchymal fibroelastosis in systemic sclerosis
T2 - Prevalence and prognostic impact
AU - Bonifazi, Martina
AU - Sverzellati, Nicola
AU - Negri, Eva
AU - Jacob, Joseph
AU - Egashira, Ryoko
AU - Moser, Joanna
AU - Piciucchi, Sara
AU - Mei, Federico
AU - De Lauretis, Angelo
AU - Visca, Dina
AU - Goh, Nicole
AU - Bonini, Matteo
AU - Cirilli, Laura
AU - La Vecchia, Carlo
AU - Chua, Felix
AU - Kouranos, Vasileios
AU - Margaritopoulos, George
AU - Kokosi, Maria
AU - Maher, Toby M.
AU - Gasparini, Stefano
AU - Gabrielli, Armando
AU - Wells, Athol U.
AU - Renzoni, Elisabetta A.
N1 - Funding Information:
Conflict of interest: M. Bonifazi reports personal fees from Boehringer Ingelheim and Roche, outside the submitted work. N. Sverzellati reports personal fees from Boehringer Ingelheim and Roche, outside the submitted work. E. Negri has nothing to disclose. J. Jacob reports personal fees from Roche and Boehringer Ingelheim, grants from GlaxoSmithKline, outside the submitted work. R. Egashira reports personal fees and non-financial support from Shionogi, Boehringer Ingelheim Japan, Bayer Yakuhin, Eisai, Daiichi Sankyo and AstraZeneca, non-financial support from Kyorin, outside the submitted work. J. Moser has nothing to disclose. S. Piciucchi has nothing to disclose. F. Mei has nothing to disclose. A. De Lauretis has nothing to disclose. D. Visca has nothing to disclose. N. Goh has nothing to disclose. M. Bonini has nothing to disclose. L. Cirilli has nothing to disclose. C. La Vecchia has nothing to disclose. F. Chua has no conflict of interest to declare V. Kouranos has nothing to disclose. G. Margaritopoulos has nothing to disclose. M. Kokosi has nothing to disclose. T.M. Maher has, via his institution, received industry-academic funding from GlaxoSmithKline R&D and UCB and has received consultancy or speakers fees from Apellis, Astra Zeneca, Bayer, Blade Therapeutics, Boehringer Ingelheim, Bristol-Myers Squibb, Galapagos, GlaxoSmithKline R&D, Indalo, Novartis, Pliant, ProMetic, Respivnat, Roche, Samumed and UCB. S. Gasparini has nothing to disclose. A. Gabrielli has nothing to disclose. A.U. Wells reports personal fees from Boehringer Ingelheim, Roche and Bayer, outside the submitted work. E.A. Renzoni reports personal fees from Boehringer Ingelheim and Roche, outside the submitted work.
Funding Information:
Support statement: This work was supported in part by Versus Arthritis and by Scleroderma and Raynaud’s UK. Joseph Jacob was funded by Wellcome Trust Clinical Research Career Development Fellowship 209553/Z/17/Z.
Publisher Copyright:
Copyright © 2020 ERS.
Copyright:
Copyright 2020 Elsevier B.V., All rights reserved.
PY - 2020/7/1
Y1 - 2020/7/1
N2 - Interstitial lung disease (ILD) in systemic sclerosis (SSc) is a major cause of morbidity and mortality, mostly presenting as non-specific 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 centres 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 four-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.
AB - Interstitial lung disease (ILD) in systemic sclerosis (SSc) is a major cause of morbidity and mortality, mostly presenting as non-specific 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 centres 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 four-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.
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U2 - 10.1183/13993003.02135-2019
DO - 10.1183/13993003.02135-2019
M3 - Article
C2 - 32299855
AN - SCOPUS:85088485500
VL - 56
JO - European Respiratory Journal
JF - European Respiratory Journal
SN - 0903-1936
IS - 1
M1 - 1902135
ER -