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Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is characterized by upper lobe-dominant fibrosis involving the pleura and subpleural lung parenchyma. Pathologically, it is characterized by parenchymal intra-alveolar fibrosis with marked deposition of elastic fibers and dense thickening of the visceral pleura. Since iPPFE was categorized as a rare idiopathic interstitial pneumonia (IIP) by the America Thoracic Society/European Respiratory Society, several studies have been conducted, revealing an overall picture of iPPFE in terms of epidemiology, clinical manifestations, and mortality, in addition to its radiological and histological characteristics.
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Interstitial lung disease (ILD), characterized by inflammation and fibrosis, often suffers from low diagnostic accuracy and consistency. Traditional hematoxylin and eosin (H&E) staining primarily reveals cellular inflammation with limited detail on fibrosis. To address these issues, we introduce a pioneering label-free quantitative multiphoton fiber histology (MPFH) technique that delineates the intricate characteristics of collagen and elastin fibers for ILD diagnosis.
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