Enomoto Yasunori, Nakamura Yutaro, Colby Thomas V, Johkoh Takeshi, Sumikawa Hiromitsu, Nishimoto Koji, Yoshimura Katsuhiro, Matsushima Sayomi, Oyama Yoshiyuki, Hozumi Hironao, Kono Masato, Fujisawa Tomoyuki, Enomoto Noriyuki, Inui Naoki, Iwashita Toshihide, Suda Takafumi
Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Shizuoka, Japan.
Department of Regenerative and Infectious Pathology, Hamamatsu University School of Medicine, Shizuoka, Japan.
PLoS One. 2017 Jun 30;12(6):e0180283. doi: 10.1371/journal.pone.0180283. eCollection 2017.
Radiologic pleuroparenchymal fibroelastosis (PPFE)-like lesion including pulmonary apical cap can be occasionally observed in clinical settings. However, the significance of radiologic PPFE-like lesion is unclear in connective tissue disease (CTD)-related interstitial lung disease (ILD).
A total of 113 patients with CTD-related ILD were enrolled and assessed for radiologic PPFE-like lesion, which was defined as bilateral, upper lobe, and subpleural dense consolidations with or without pleural thickening on chest high-resolution computed tomography. The clinical, radiologic, and pathologic characteristics were evaluated.
Radiologic PPFE-like lesion was found in 21 patients (19%) and were relatively frequent in those with systemic sclerosis (6/14: 43%) and primary Sjögren's syndrome (4/14: 29%). Patients with PPFE-like lesion were significantly older, had lower body mass index, higher ratio of residual volume to total lung capacity, and higher complication rate of pneumothorax and/or pneumomediastinum than those without. Twelve of the 21 patients were diagnosed radiologically as usual interstitial pneumonia (UIP) or possible UIP pattern. Two of three patients who underwent surgical lung biopsy of the upper lobes showed UIP on histopathology. Another patient was confirmed to have upper lobe PPFE on autopsy. During the clinical course, progression of the radiologic PPFE-like lesions was observed in 13 of 21 patients. Six patients died (mortality rate: 29%) and their PPFE-like lesions were commonly progressive. In the total cohort, our multivariate analysis identified the presence of PPFE-like lesion as a significant risk factor for respiratory death (hazard ratio: 4.10, 95% confidence interval: 1.33-12.65, p = 0.01).
In patients with CTD-related ILD, radiologic PPFE-like lesion, which may present as not only PPFE but also apical cap and upper lobe subpleural fibrosis commonly due to UIP, was not uncommon and was associated with poor prognosis. Clinicians should be cautious with this radiologic finding, particularly when it is progressive.
在临床环境中偶尔可观察到包括肺尖帽在内的放射学胸膜实质纤维弹性组织增生症（PPFE）样病变。然而，在结缔组织病（CTD）相关的间质性肺疾病（ILD）中，放射学PPFE样病变的意义尚不清楚。
共纳入113例CTD相关ILD患者，评估其放射学PPFE样病变，该病变定义为胸部高分辨率计算机断层扫描显示双侧、上叶及胸膜下致密实变，伴或不伴胸膜增厚。对临床、放射学和病理学特征进行评估。
21例患者（19%）发现有放射学PPFE样病变，在系统性硬化症患者（6/14：43%）和原发性干燥综合征患者（4/14：29%）中相对常见。与无PPFE样病变的患者相比，有PPFE样病变的患者年龄显著更大，体重指数更低，残气量与肺总量之比更高，气胸和/或纵隔气肿的并发症发生率更高。21例患者中有12例经放射学诊断为普通型间质性肺炎（UIP）或可能的UIP型。接受上叶手术肺活检的3例患者中有2例组织病理学显示为UIP。另一例患者尸检证实有上叶PPFE。在临床过程中，21例患者中有13例观察到放射学PPFE样病变进展。6例患者死亡（死亡率：29%），其PPFE样病变通常呈进行性。在整个队列中，我们的多变量分析确定PPFE样病变的存在是呼吸死亡的一个重要危险因素（风险比：4.10，95%置信区间：1.33 - 12.65，p = 0.01）。
在CTD相关ILD患者中，放射学PPFE样病变不仅可表现为PPFE，还可表现为肺尖帽和通常由UIP引起的上叶胸膜下纤维化，并不罕见，且与预后不良相关。临床医生应对这一放射学表现保持谨慎，尤其是当其呈进行性时。
