Traction bronchiectasis and platythorax on computed tomography are determinants of progression and mortality in pleuro-parenchymal fibroelastosis

Abstract

Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial disease characterised by accumulation of elastin-rich fibrosis and predominant upper lobe shrinkage. The pathogenesis is poorly understood but idiopathic and secondary forms are recognised; an association with Aspergillus infection has been reported. We evaluated computed tomography (CT) and clinical/serological indices to identify factors influencing progression and mortality in patients with PPFE. CT studies in 72 patients, with a multidisciplinary diagnosis of PPFE, were consensus scored by two thoracic radiologists. The presence and severity of traction bronchiectasis in areas of PPFE correlated with the extent, severity and progression of PPFE (all p 40 mgA/L) was seen in 18/72 (25%) patients and was higher than in reference cohorts of idiopathic pulmonary fibrosis (20/105; 19%) and chronic hypersensitivity pneumonitis (12/111; 11%) (p=0.04). These data highlight the importance of platythorax as a clinico-radiological marker of volume loss and, more importantly, an index of poor outcome in PPFE, potentially reflecting inexorable physiological decline. The possibility that fungal sensitisation may form part of a pathogenetic or progressive PPFE profile warrants further investigation.