Pleuroparenchymal fibroelastosis in idiopathic pulmonary fibrosis: Survival analysis using visual and computer-based computed tomography assessment

Abstract

Please cite this article as: E. Gudmundsson et al., Pleuroparenchymal fibroelastosis in idiopathic pulmonary fibrosis: Survival analysis using visual and computer-based computed tomography assessment, EClinicalMedicine (2021),

Delineating associations of progressive pleuroparenchymal fibroelastosis in patients with pulmonary fibrosis

“…14,15 Computer quantitation of baseline upper lobe PPFE-like lesions (incidence 25-36%) was shown to associate with mortality in IPF independent of baseline disease severity (measured by either FVC, ILD extent or diffusion capacity for carbon monoxide [DLco]) and identified more patients with a poor outcome than equivalent semi-quantitative visual CT analysis. 11 PPFE-like lesions scored by computer did not associate with baseline measures of IPF-related fibrosis on univariable or multivariable analyses, suggesting that PPFE-related damage might represent injury occurring independent to IPFrelated lung fibrosis. 11 Careful delineation of patients with clinically meaningful progressive PPFE-like lesions using computational analysis of time-series CTs may highlight a lung fibrosis endotype benefiting from alternative management strategies.…”

“…Exploration of novel imaging features has also delineated patterns such as pleuroparenchymal fibroelastosis (PPFE) which do not directly result from the fibrotic process, but which may influence patient survival. 11 PPFE is characterised by dense triangular pleurally-based opacities occurring in the upper lobes on CT. 12 PPFE scored visually on a single baseline CT has been shown to associate with reduced survival time in IPF patients 11,13 and other fibrosing lung diseases. 14,15 Computer quantitation of baseline upper lobe PPFE-like lesions (incidence 25-36%) was shown to associate with mortality in IPF independent of baseline disease severity (measured by either FVC, ILD extent or diffusion capacity for carbon monoxide [DLco]) and identified more patients with a poor outcome than equivalent semi-quantitative visual CT analysis.…”

Pleuroparenchymal fibroelastosis

“…Rare genetic variants, especially telomere-related gene mutations, were identified in patients with PPFE, some of them with a family history of ILD [20,21]. Similarly, a pattern of PPFE in the upper lung zones may be associated with a usual interstitial pneumonia (UIP) pattern in the lung bases [22–24,25 ▪▪ ,26 ▪▪ ], therefore with often unclear boundaries between idiopathic pulmonary fibrosis (IPF) with PPFE features versus PPFE combined with UIP pattern.…”

“…The PPFE with UIP pattern group showed a significantly higher RV, higher PaCO 2 , higher complication rate of pneumothorax and pneumomediastinum, and a trend towards shorter survival compared with patients with IPF/UIP [22]. Gudmundsson et al [26 ▪▪ ] visually scored HRCTs for PPFE features (absent, moderate, marked) in patients with IPF. PPFE features were present in 49% of 142 patients in the derivation cohort and 72% of 145 patients in the validation cohort.…”

“…PPFE features were present in 49% of 142 patients in the derivation cohort and 72% of 145 patients in the validation cohort. Marked PPFE present in 10.8% of patients was associated with 1-year FVC decline and mortality [26 ▪▪ ]. Fujisawa et al [25 ▪▪ ] retrospectively identified PPFE features (PPFE-like lesions) in 24.1% of 419 patients with idiopathic interstitial pneumonia, mainly IPF and unclassifiable fibrotic ILD.…”