Pleuroparenchymal Fibroelastosis: Obstacles and Challenges
Deadline for manuscript submissions: closed (30 June 2021) | Viewed by 14374
The field of pleuroparenchymal fibroelastosisis (PPFE) research is growing rapidly. PPFE is a rare interstitial pneumonia consisting of alveolar septal elastosis and intra-alveolar collagenosis, predominantly located in the upper lobes. Recent discoveries suggest that the clinical and physiological characteristics of upper lobe fibroelastosis differ from those of idiopathic pulmonary fibrosis (IPF), including a flattened thoracic cage and a marked decrease in forced vital capacity, but with preserved residual volume. The prognosis may be related to the development of fibrosing interstitial pneumonia in the lower lobes. However, there is marked variation in the pathogenesis and clinical features of PPFE. Although the diagnostic criteria for PPFE are based on histological findings by surgical lung biopsy (SLB), we have limited chances to perform an SLB for the diagnosis of PPFE. A proposal concerning the diagnostic criteria for idiopathic PPFE with and without an SLB was recently published. However, there are no established blood biomarkers to aid in the diagnosis or prediction of a progressive phenotype in PPFE patients. Mechanisms of the occurrence and evolution of fibroelastosis in PPFE are still unknown. In addition, treatment strategies for PPFE, targeting the inhibition of elastosis, have not been established. The present Special Issue aims to deepen our understanding of the characteristics of PPFE to increase our knowledge of a, so far, poorly explored research area, and to seek innovative treatment of PPFE.
Prof. Dr. Hiroshi Ishii
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- peuroparenchymal fibroelastosis
- clinical and physiological findings
- prognostic predictor
- histological characteristics
- predisposing factors or comorbidities
- pathogenic mechanism
- biomarker/molecular target