Pleuroparenchymal Fibroelastosis: Clinical Recognition, Risk Stratification, and Management in the Era of Cancer Survivorship

Abstract Purpose of Review This review highlights advances in the diagnosis, risk stratification, and management of pleuroparenchymal fibroelastosis (PPFE), emphasizing its growing recognition as a late complication of cancer therapy and a progressive pattern of fibrotic lung remodeling. Recent Findings PPFE is increasingly recognized across a broad range clinical settings, supporting a shared fibroelastotic response to chronic lung injury. In oncologic survivorship populations, prolonged latency contributes to delayed recognition. Recent studies using quantitative imaging have improved characterization of structural progression and further demonstrated discordance between radiographic disease burden and physiologic measures. Emerging assessment frameworks propose integrating radiographic progression, nutritional status, and clinical trajectory into longitudinal disease evaluation. Summary Early recognition of PPFE remains challenging. The growing population of pediatric cancer survivors reframes PPFE as a late effect of prior therapy with important implications for long-term surveillance and transition of care. Improved, multidimensional frameworks may improve prognostication and help guide management strategies.