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 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).
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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 … WebPleuroparenchymal fibroelastosis (PPFE) is an unusual pulmonary disease with unique clinical, radiological, and pathological characteristics. Designated a rare idiopathic … space needed for stove
Idiopathic Pleuroparenchymal Fibroelastosis - PubMed
WebJul 7, 2016 · Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disease, idiopathic in most of the cases described in the literature. We report the case of a 55-year-old patient, non-smoker with tuberculosis treated in his youth, starting with progressive dyspnoea and cough, with radiographic abnormalities consisting of pleural thickening, … WebJul 1, 2024 · The median FVC in enrolled patients with iPPFE was 54.5% at the initiation of nintedanib treatment, indicating that patients presented relatively advanced iPPFE. Therefore, patients with iPPFE in this study who exhibited a rapid decline in FVC while receiving nintedanib could simply be experiencing the endpoint of the slow deterioration … WebApr 8, 2024 · Combined pulmonary fibrosis and emphysema is a controversial and poorly defined entity. Smoking-related lung diseases can coexist and the combination of … teams owa usmc