Pleuroparenchymal fibroelastosis in patients with idiopathic pulmonary fibrosis

Abstract

Background and objective PPFE is characterized by fibrosis in the pleura and subpleural lung parenchyma in the upper lobes, while other types of ILD, mainly UIP, can be observed in about half of the patients in their lower lobes. The aim of this study was to evaluate the clinical significance of the radiologically defined PPFE in patients with IPF. Methods Clinical data and chest CT images were retrospectively analysed in 445 patients with IPF (biopsy‐proven cases, n = 165). The radiological criteria of PPFE were defined as follows: (i) bilateral subpleural dense fibrosis with or without pleural thickening in the upper lobes, (ii) evidence of disease progression and (iii) no clinical evidence of identifiable aetiologies. Results The median follow‐up period was 43.0 months. The mean age of the patients was 66.4 years and 76.4% were male. PPFE was identified in 28 patients (6.3%). The PPFE group showed lower BMI and lung function (FVC and TLC) at baseline, more frequent pneumothorax and pneumomediastinum, higher decline rates in lung function and poorer prognosis during follow‐up than the no‐PPFE group. PPFE was an independent risk factor (HR = 2.953, 95% CI: 1.350–6.460, P = 0.007) for pneumothorax or pneumomediastinum, but not for mortality in patients with IPF. Conclusion Among patients with IPF, the PPFE group, when compared to the no‐PPFE group, showed lower BMI and lung function and showed more frequent complications and poorer survival during follow‐up.

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

“…However, in neither study was PPFE associated with accelerated FVC decline. In a recent Korean study of IPF patients, PPFE was identified in 6·3% of patients suggesting that geographic and/or genetic factors may influence PPFE prevalence [5] . In the Korean cohort, PPFE was associated with increased FVC decline on univariable analyses alone, and no beneficial impact of antifibrotic medication was seen on mortality in the limited cohort of patients with PPFE.…”

“…PPFE has been increasingly reported in association with IPF in limited series from single centres. Greater extents of visually-scored PPFE have been associated with worsened prognosis [ 4 , 5 ]. An increase in visually-scored PPFE was associated with greater rapid forced vital capacity (FVC) decline in a single report in a large IPF series [6] .…”

Prognostic and Clinical Value of Cluster Analysis in Idiopathic Pleuroparenchymal Fibroelastosis Phenotypes

“…Oda et al reported that the survival time of PPFE with the UIP pattern tended to be shorter than that of IPF [19]. However, Lee et al recently reported radiologic findings suggesting that PPFE was an independent risk factor for pneumothorax or pneumomediastinum, except for mortality in patients with IPF [20]. Meanwhile, in our previous study with a small cohort, there were no significant differences in prognosis between patients with idiopathic PPFE who presented with lowerlobe UIP/possible UIP pattern and those without.…”

Treatment with antifibrotic agents in idiopathic pleuroparenchymal fibroelastosis with usual interstitial pneumonia

“…Japan from April 2003 to May 2018. The diagnosis of IPPFE with UIP, which was modified from previous studies [3,15,16], was determined by the presence of the following features in addition to honeycombing, predominantly in the bilateral lower lobes: (i) bilateral dense subpleural consolidation in the upper lobes with traction bronchiolectasis, architectural distortion, and upper lobe volume loss; ii) exclusion of other identifiable aetiologies, such as a history of radiation therapy, active pulmonary infection, connective tissue disease and chronic hypersensitivity pneumonitis and (iii) radiologic confirmation of disease progression (defined as an increase in the upper lobe subpleural consolidation and/or a decreased in upper lobe volume on serial chest high-resolution computed tomography (HRCT). We identified IPF in accordance with the 2011 international IPF guidelines [17], and acute exacerbation of IPF was diagnosed by criteria proposed by Collard et al [18], in which all of the following 4 conditions must be satisfied: (i) a previous or concurrent diagnosis of (every 6 months) and prognosis.…”