Pleuroparenchymal fibroelastosis in patients with idiopathic pulmonary fibrosis. Issue 10 (9th March 2020)

• Record Type: Journal Article
• Authors: Lee, Song‐I.; Chae, Eun Jin; Song, Joon Seon; Lee, Jae Ha; Song, Jin Woo
• Is Part Of: Respirology. Volume 25:Issue 10(2020)
• Page Start: 1046
• Page End: 1052
• Publication Date: 2020-03-09
• Subjects: fibroelastosis -- idiopathic pulmonary fibrosis -- interstitial lung disease -- prognosis -- radiological diagnostic; Respiratory organs -- Diseases -- Periodicals; Respiratory organs -- Periodicals; 612.2
• DOI: 10.1111/resp.13796
• Languages: English
• ISSNs: 1323-7799

Abstract

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. Abstract : Radiological PPFE was identified in 6.3% of patients with IPF. Radiological PPFE was associated with low BMI and lung function at baseline, more frequent pneumothorax and pneumomediastinum, higher decline rates in lung function and poorer prognosis during follow‐up in patients with IPF.