Suspected pleuroparenchymal fibroelastosis relapse after lung transplantation: a case report and literature review

Pleuroparenchymal fibroelastosis (PPFE) is a very rare lung disease characterized by dense fibrous thickening of the visceral pleura and intraalveolar fibrosis containing prominent elastosis, with typical upper lobe predominance. PPFE usually shows progressive restrictive ventilatory impairment refractory to medical treatment; bilateral lung transplantation currently remains the only available therapeutic option. In this report, we describe a case of suspected PPFE relapse after lung transplantation that, to our knowledge, has never been described in the medical literature. A 48-year-old male with idiopathic pleuroparenchymal fibroelastosis underwent a bilateral lung transplant in our department. 8 months later, he presented with progressively worsening clinical condition, his respiratory state gradually deteriorated. High-resolution CT again showed bilateral diffused parenchymal consolidations, with prevalence in the upper lobes and subpleural regions. A PPFE relapse was therefore suspected, so he was listed for lung retransplantation, which was performed ten months after the first transplant. Histopathological analysis of the second explanted lung again confirmed the diagnosis of PPFE. The case highlights the possibility of PPFE relapse after lung transplantation, that may add to the increasing evidence of an underlying auto-immune mechanism contributing to its pathogenesis.