Description:
<jats:p><jats:styled-content style="fixed-case">PPFE</jats:styled-content> is a rare disease characterized by upper lobe pleural fibrosis and parenchymal fibroelastosis. Its aetiology is considered idiopathic, although possible causative factors have been described. An association of <jats:styled-content style="fixed-case">PPFE</jats:styled-content> with solid tumours is unknown and has not been considered previously. We identified six patients with <jats:styled-content style="fixed-case">PPFE</jats:styled-content>, four of them with a coexisting malignancy. The case series suggests that <jats:styled-content style="fixed-case">PPFE</jats:styled-content> might be an implication of varying factors rather than being an exclusively idiopathic condition.</jats:p>