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Upper‐lobe predominance of elastofibrosis is agreed upon for the diagnosis of clinical pleuroparenchymal fibroelastosis (PPFE). We herein describe a patient with dermatomyositis‐related interstitial pneumonia with a histology of lower‐lobe predominant PPFE. A 71‐year‐old woman who had been diagnosed with dermatomyositis‐related interstitial pneumonia died of respiratory failure. The computed tomography...
Fibroblastic foci, a major histological feature of usual interstitial pneumonia (UIP), play a critical role in the development of UIP. The mechanisms involved in the formation of these foci, however, including cellular origin, remain unclear. Recent in vitro and animal studies suggested epithelial–mesenchymal transition (EMT) of alveolar epithelial cells during pulmonary fibrogenesis. The aim of the...
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