A Case of Pleuroparenchymal Fibroelastosis

Abstract

Pleuroparenchymal fibroelastosis (PPFE) is a rare fibrotic lung disease with a poor prognosis. Some patients with PPFE have prominent lesions in one upper lobe. Due to the unilateral nature of lesions, similarities between some patients of PPFE and unilateral upper field pulmonary fibrosis (unilateral upper-PF) have been indicated. A 55-year-old man was referred to our hospital with PPFE, which had developed dyspnea 9 months previously. He had undergone resection of renal cancer 64 months prior to the onset of PPFE. He was administered the antifibrotic drug nintedanib, but passed away due to respiratory failure 43 months after the onset. What were impressive points in the clinical course of this PPFE patient was the similarity to unilateral upper-PF, including his medical history, and the poor prognosis. Although very rare, we do believe that the information on medical history and progression in this patient might provide suggestion into the treatment of future patients with a similar trajectory.