Radiological pleuroparenchymal fibroelastosis-like lesion in idiopathic interstitial pneumonias

Abstract

Background Pleuroparenchymal fibroelastosis (PPFE) is characterised by predominant upper lobe pleural and subpleural lung parenchymal fibrosis. Radiological PPFE-like lesion has been associated with various types of interstitial lung diseases. However, the prevalence and clinical significance of radiological PPFE-like lesion in patients with idiopathic interstitial pneumonias (IIPs) are not fully understood. We aimed to determine the prevalence and clinical impact on survival of radiological PPFE-like lesion in patients with IIPs. Methods A post-hoc analysis was conducted using data from the Japanese nationwide cloud-based database of patients with IIPs. All the patients in the database were diagnosed as having IIPs by multidisciplinary discussion. Patients diagnosed with idiopathic PPFE were excluded. Clinical data and chest computed tomography (CT) image of 419 patients with IIPs were analysed. The presence of radiological PPFE-like lesion was independently evaluated by two chest radiologists blind to the clinical data. Results Of the 419 patients with IIPs, radiological PPFE-like lesions were detected in 101 (24.1%) patients, mainly in idiopathic pulmonary fibrosis (IPF) and unclassifiable IIPs, but less in idiopathic nonspecific interstitial pneumonia. Prognostic analyses revealed that radiological PPFE-like lesion was significantly associated with poor outcome in patients with IIPs, which was independent of age, IPF diagnosis and %FVC. In survival analyses, the patients with radiological PPFE-like lesions had poor survival compared with those without (log-rank, p < 0.0001). Subgroup analyses demonstrated that radiological PPFE-like lesion was significantly associated with poor survival both in patients with IPF and those with unclassifiable IIPs. Conclusion Radiological PPFE-like lesion is a condition that could exist in IIPs, mainly in IPF and unclassifiable IIPs. Importantly, the radiological PPFE-like lesion is a non-invasive marker to predict poor outcome in patients with IIPs, which should be carefully considered in clinical practice.

Prognostic significance of radiological pleuroparenchymal fibroelastosis inMycobacterium aviumcomplex lung disease: a multicentre retrospective cohort study

“…Radiological PPFE has also been reported to be associated with poor prognosis in IPF and collagen diseases, such as scleroderma 12 18 33. As mentioned earlier, the presence of radiological PPFE was associated with low BMI.…”

“…27 Radiological PPFE was defined as bilateral, upper lobe and subpleural dense consolidations with traction bronchiectasis below the area within 1 cm from the apex of the lung on HRCT, with a minimum width of 1 cm in contact with the pleura, and distinguished from PAC (figure 2B). 12 18 21 28 The HRCT pattern of MAC lung disease was classified as nodular bronchiectatic, fibrocavitary or indeterminate 2 26. ILD findings were defined as bilateral reticular opacities with/without honeycombing on chest HRCT.…”

“…Patients with PPFE have unique chest high-resolution CT (HRCT) findings characterised by dense subpleural consolidation with traction bronchiectasis and architectural distortion, primarily in the upper lobes of the lungs 13–15. Such HRCT features, which are radiological PPFE, coexisted in patients with an IIP other than idiopathic PPFE and those with various pulmonary diseases, including hypersensitivity pneumonitis 10 16–18. An association between PPFE and recurrent respiratory infections has also been suggested; however, evidence regarding the association between PPPE and MAC lung disease is insufficient 16 19.…”

Pleuroparenchymal fibroelastosis

“…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. Radiological PPFE features were significantly associated with a poor survival, including in subgroup analyses in patients with IPF and those with unclassifiable IIPs [25 ▪▪ ].…”

Effects of Automatic Deep-Learning-Based Lung Analysis on Quantification of Interstitial Lung Disease: Correlation with Pulmonary Function Test Results and Prognosis

“…Thus, we have developed a fully automatic deep-learning (DL)-based lung analysis model that can differentiate consolidation with fibrosis, such as pleuroparenchymal fibroelastosis (PPFE)-like lesions, from consolidation without fibrosis. As the PPFE-like lesions are associated with ILD severity [ 12 , 13 , 14 , 15 , 16 ], it is crucial to distinguish and separately quantify consolidation with and without fibrosis. To our knowledge, no other DL software or CAD systems have this ability [ 10 , 17 , 18 , 19 ].…”