Article
Integrated phenotyping identifies reproducible prognostic subgroups in idiopathic pleuroparenchymal fibroelastosis.
Respiratory research - 26 Jun 2026
Otoshi Ryota, Kitamura Hideya, Oda Tsuneyuki, Kanzawa Akihiro, Okamura Ken, Niwa Takashi, Baba Tomohisa, Iwasawa Tae, Takemura Tamiko, Mizuguchi Kenji, Natsume-Kitatani Yayoi, Ogura Takashi
Abstract excerpt
BACKGROUND: Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease characterized by upper-lobe-predominant fibrosis and heterogeneous prognosis. An integrated phenotype classification incorporating clinical, radiological and biological markers has not been established. METHODS: We conducted a retrospective observational study of 32 patients with idiopathic PPFE in an exploratory...
Read the complete abstract on PubMedTopics
- Humans
- Female
- Phenotype
- Retrospective Studies
- Male
- Prognosis
