pulmonary disorders
Idiopathic Pleuroparenchymal Fibroelastosis
Prognosis for Idiopathic PPFE
The clinical course in patients with idiopathic pleuroparenchymal fibroelastosis tends to be progressive in the majority of patients. Disease progression occurs in most patients with 5 year survival rates approximately 25 to 60% ( Prognosis reference ). 1.Kinoshita Y, Ikeda T, Miyamura T, et al. A proposed prognostic prediction score for pleuroparenchymal fibroelastosis.Respir Res22: 215, 2021. https://doi.org/10.1186/s12931-021-01810-z 