Pleuroparenchymal fibroelastosis in systemic sclerosis: Prevalence and prognostic impact

Martina Bonifazi, Nicola Sverzellati, Eva Negri, Joseph Jacob, Ryoko Egashira, Joanna Moser, Sara Piciucchi, Federico Mei, Angelo De Lauretis, Dina Visca, Nicole Goh, Matteo Bonini, Laura Cirilli, Carlo La Vecchia, Felix Chua, Vasileios Kouranos, George Margaritopoulos, Maria Kokosi, Toby M. Maher, Stefano GaspariniArmando Gabrielli, Athol U. Wells, Elisabetta A. Renzoni

Research output: Contribution to journalArticlepeer-review


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; 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.

Original languageEnglish
Article number1902135
JournalEuropean Respiratory Journal
Issue number1
Publication statusPublished - Jul 1 2020

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine


Dive into the research topics of 'Pleuroparenchymal fibroelastosis in systemic sclerosis: Prevalence and prognostic impact'. Together they form a unique fingerprint.

Cite this