WebPPFE is a rare, idiopathic interstitial pneumonia with specific clinical, radiological, and pathological features. It affects the visceral pleura and the subpleural parenchyma with an upper lobe predilection and can be associated with a separate interstitial lung disease (ILD). WebPleuroparenchymal Fibroelastosis (PPFE) is a rare inter- stitial lung disease that is characterized by upper lobes pleural thickening and subpleural fibrosis.1Differently from other more common patterns of pulmonary fibrosis, like usual interstitial pneumonia (UIP) and non-specific interstitial pneumonia (NSIP), PPFE typically shows prominent …
Treatment with antifibrotic agents in’idiopathic …
Pleuroparenchymal fibroelastosis (PPFE) is a rare, benign entity. About half of cases are felt to be idiopathic, with the other half secondary to underlying diseases or conditions (e.g. transplantation). Idiopathic cases belong to the group of idiopathic interstitial pneumonias. Epidemiology See more Pleuroparenchymal fibroelastosis is considered a rare disorder; however, it is very likely both under-recognized and misdiagnosed … See more The pathophysiology is unknown. Pleuroparenchymal fibroelastosis is characterized by predominantly upper … See more Most often significant chronic respiratory symptoms, which may include: 1. non-productive cough 2. dyspnea, either on exertion or in a worsening course 3. angina 4. frequent … See more Chest radiographs may be normal or present non-specific findings comprising: 1. marked bilateral apical pleural thickening 2. … See more WebNov 22, 2024 · Watanabe et al. [] recently proposed diagnostic criteria of IPPFE that describe four categories based on the clinical symptoms, radiology, histology, and physiology: “definite IPPFE”, “radiologically and physiologically probable IPPFE”, “radiologically probable IPPFE”, and “radiologically possible IPPFE”.A noteworthy feature of the diagnostic criteria … flowers lemon grove
Criteria for the diagnosis of idiopathic pleuroparenchymal ...
WebOct 14, 2024 · Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare lung disease that manifests as parenchymal fibrosis of the upper lung lobe and pleura. There have been no reports of IPPFE complicating pregnancy. Here, we report a case of IPPFE that deteriorated rapidly during pregnancy. Case presentation WebThe three diagnostic criteria for IPPFE without SLB are as follows: (1) radiologically possible IPPFE (a radiological criterion confirming CT lesions in both lung apexes, regardless of … WebJul 1, 2024 · We propose the following three criteria for the diagnosis of “definite IPPFE” in patients who have undergone SLB: (1) Multiple subpleural areas of airspace consolidation, with traction bronchiectasis located predominantly in the upper lobes, as visualized on high-resolution computed tomography (HRCT) scans ( Fig. 1) [10], [13], [14], [15], [16]. flowers leis are made from