1 Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Fukuoka, Japan.
2 Department of Pathology, Fukuoka University Hospital, Fukuoka, Japan.
Chron Respir Dis. 2019 Jan-Dec;16:1479973119867945. doi: 10.1177/1479973119867945.
The idiopathic form of pleuroparenchymal fibroelastosis (PPFE) is categorized as a rare idiopathic interstitial pneumonia in the current classification. The majority of PPFE cases are idiopathic, but many predisposing factors or comorbidities have been reported. Although histological PPFE is predominantly located in the upper lobes, which are less often affected by fibrosis in patients with idiopathic pulmonary fibrosis (IPF), the clinical course of PPFE is seemingly similar to that of IPF. However, upper lobe fibroelastosis has various clinical and physiological characteristics that differ from those of IPF, including a flattened thoracic cage and a marked decrease in the forced vital capacity (FVC) but with a preserved residual volume. Compared with IPF, the decrease in the walking distance is mild despite the markedly decreased FVC in PPFE, and chest radiograph more frequently shows the elevation of bilateral hilar opacities with or without tracheal deviation. The prognosis may be related to the development of fibrosing interstitial pneumonia in the lower lobes with elevated levels of serum Krebs von den Lungen-6; however, there is marked variation in the pathogenesis and clinical features in PPFE. A proposal of the diagnostic criteria for idiopathic PPFE with and without surgical lung biopsy, which has recently been published, may be useful.
特发性胸膜肺弹力纤维增生症（PPFE）的特发型被归类为当前分类中的一种罕见特发性间质性肺炎。大多数 PPFE 病例是特发性的，但已有许多诱发因素或合并症被报道。尽管组织学上的 PPFE 主要位于上叶，而上叶在特发性肺纤维化（IPF）患者中较少受到纤维化的影响，但 PPFE 的临床过程似乎与 IPF 相似。然而，上叶弹力纤维增生症具有与 IPF 不同的各种临床和生理特征，包括胸廓平坦和用力肺活量（FVC）明显下降，但残气量保留。与 IPF 相比，尽管 PPFE 的 FVC 明显下降，但步行距离的下降较轻，并且胸部 X 线更频繁地显示双侧肺门抬高，伴有或不伴有气管偏移。预后可能与血清 Krebs von den Lungen-6 水平升高的下叶纤维化间质性肺炎的发展有关；然而，PPFE 的发病机制和临床特征存在明显差异。最近发表的特发性 PPFE 有和无外科肺活检的诊断标准的建议可能是有用的。
