S123 Pleuroparenchymal fibroelastosis: clinical, functional and morphologic determinants of mortality

Introduction and Objectives Progressive pleuroparenchymal fibroelastosis (PPFE) is associated with a high symptom burden and frequently co-exists with a separate interstitial lung disease. The prognostic impact of such combinations is unclear and the clinical and computed tomographic (CT) determinants of mortality remain poorly characterised. Methods Patients with a diagnosis of PPFE (2004–19) were retrieved from the Royal Brompton Hospital ILD databases. CTs were evaluated for radiologic features, including: 1) the cranio-caudal extent and the severity of PPFE; 2) the hilar position (ratio of the lung apex to the ‘hilar point’ distance/lung apex to the diaphragmatic dome distance); 3) upper lobe volume loss, and 4) presence of co-existent ILD. Results 139 patients (75 [54%] female; median age 63.5, IQR 52–71.5) were evaluated, including 51 (36.7%) with idiopathic PPFE, 41 (29.5%) with concomitant idiopathic UIP, 17 (12.2%) with hypersensitivity pneumonitis and 8 (5.8%) with autoimmunity. Histopathological information was available in 50 (36%) patients, including from 39 surgical biopsies. 51 deaths were recorded among 130 patients with longitudinal data, yielding a median survival of 3 years. The mean severity of PPFE was negatively correlated with hilar position (r = -0.38, P Conclusions Patients with progressive PPFE have a poor outcome, with a median survival that is comparable to IPF. Identifiable and measurable changes in specific clinical, physiologic and radiologic parameters appear to characterise the adverse prognostic profile of these individuals.