2024年6月28日 · Pleuroparenchymal fibroelastosis is characterized by predominantly upper lobe pleural and subjacent parenchymal fibrosis (the latter being intra-alveolar with accompanying elastosis of the alveolar walls). The etiology remains unknown. Familial cases have been reported, especially in young women 8.

The principal and ancillary CT findings of PPFE have been highlighted in multiple studies (17, 27, 38, 47, 48, 50). For diagnostic purposes, cases should be examined using both standard axial and coronal reconstruction images ( Figure 2 ).

2025年3月13日 · Pleuroparenchymal fibroelastosis (PPFE) is an interstitial lung disease (ILD) characterized at CT by upper lobe–predominant pleural thickening and subpleural fibrosis and histologically by visceral pleural fibrosis and subpleural fibroelastosis.

Reddy and coworkers proposed CT criteria for “definite” PPFE, including upper lobe pleural thickening with subpleural fibrosis and limited, if any, lower lobe involvement . Tractional distortion of the airways within areas of PPFE is common, reflecting the dense surrounding fibrosis of the disease ( 38 , 47 , 48 ).

Pleuroparenchymal fibroelastosis (PPFE) is a rare pulmonary fibrosis that is clinically characterized by upper-lobe predominant fibrosis. PPFE is a slowly progressive disorder and its first symptom is dyspnea or dry cough. Chest pain because of pneumothorax may be the first symptom in some patients.

2023年11月20日 · CT scan. Apices are notable for: Pleural thickening, with dense subpleural consolidation. Traction bronchiectasis. There is clear demarcation between fibrotic lung and unaffected lung. In advanced disease, honeycombing and bullae may occur (which explains the tendency to develop pneumothorax). Shifts in thoracic geometry:

2022年1月1日 · Pleuroparenchymal fibroelastosis (PPFE) is a rare, generally idiopathic form of interstitial pneumonia with unique clinical, radiological and histopathological features. It is named after the presence of upper lobe pleural and subjacent parenchymal fibrosis, with …

Pleuroparenchymal fibroelastosis (PPFE) is an interstitial lung disease (ILD) characterized at CT by upper lobe-predominant pleural thickening and subpleural fibrosis and histologically by visceral pleural fibrosis and subpleural fibroelastosis.

2022年6月22日 · 胸膜肺实质弹性纤维增生症（PPFE）是间质性肺病（ILD）的一种形态学模式，表现为胸膜下肺不张硬结伴弹性纤维增生和胸膜纤维增厚，类似于组织学上的“心根尖帽纤维化 [1] ”。 PPFE可能呈特发性，或与造血干细胞移植后、结缔组织疾病和其他类型的ILD相关 [2] 。 日本患者报告的特发性上肺叶肺纤维化也属于这一类别 [3] 。 尽管几十年前发表的论文 [4-10] 中出现了一些类似于PPFE的概念，但自从Frankel等人发表了第一篇命名为PPFE的论文 [11] 以 …

PPFE can be seen in the context of including infections, post-transplant, and sometimes, or association with connective tissue diseases (CTDs). When in seen with CTDs, PPFE has been reported commonly in patients with systemic sclerosis, followed by rheumatoid arthritis, and to a lesser extent, Sjogren syndrome, inflammatory myopathies, and ANCA ...