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Pleuroparenchymal fibroelastosis (PPFE) is characterized by fibrosis involving the pleura
and subpleural lung parenchyma, predominantly in the upper lobes. As PPFE appears to …

The world of rare interstitial lung diseases (ILDs) is diverse and complex. Diagnosis and
therapy usually pose challenges. This review describes a selection of rare and ultrarare …

Objectives To compare the lung CT volume (CT vol) and pulmonary function tests in an
interstitial lung disease (ILD) population. Then to evaluate the CT vol loss between …

Background The antifibrotic agent nintedanib has been reported to effectively prevent the
decline in forced vital capacity (FVC) in a broad range of interstitial lung diseases. However …

Background Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is a rare interstitial lung
disease characterised by predominant upper-lobe fibrosis involving the pleura and …

Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is characterized by upper lobe-
dominant fibrosis involving the pleura and subpleural lung parenchyma. Pathologically, it is …

Pleuroparenchymal fibroelastosis (PPFE) is a severe clinical entity that was only identified at
the turn of the 21st century. There are so many unanswered questions and clinical …

Materials and methods We retrospectively reviewed the medical records of the Department
of Respiratory Medicine of Fukuoka University Chikushi Hospital and selected consecutive …

Background Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is characterised by upper
lobe-dominant fibrosis involving the pleura and subpleural lung parenchyma, with advanced …