Sumikawa Hiromitsu, Johkoh Takeshi, Egashira Ryoko, Sugiura Hiroaki, Yamano Yasuhiko, Kataoka Kensuke, Kondoh Yasuhiro, Arakawa Hiroaki, Nakamura Masahisa, Kuriu Akihiro, Nakanishi Katsuyuki, Tomiyama Noriyuki
Department of Radiology, Sakai City Medical Center, Japan.
Department of Radiology, Osaka International Cancer Institute, Japan.
Eur J Radiol Open. 2020 Dec 11;7:100298. doi: 10.1016/j.ejro.2020.100298. eCollection 2020.
The present study aimed to evaluate the significance of Pleuroparenchymal fibroelastosis (PPFE)-like lesions in predicting prognosis in patients with chronic interstitial pneumonia (IP).
The present study enrolled 207 patients with IP in whom surgical lung biopsy was performed. Among the patients enrolled in the present study, 77 had idiopathic pulmonary fibrosis (IPF), 15 had nonspecific interstitial pneumonia (NSIP), 13 had chronic hypersensitivity pneumonitis (CHP), 41 had connective tissue disease (CTD), three had PPFE, and 58 had unclassifiable diagnosis. The incidence, characteristics, and thickness of PPFE-like lesions were evaluated in each patient with IP. Additionally, the influence of PPFE-like lesions on the prognosis was also determined.
Of 207 patients, 160 (77.3 %) showed PPFE-like lesions. The frequency of PPFE-like lesions was similar in patients with IPF, NSIP, CHP, CTD, and unclassifiable diagnosis (79.5 %, 79.5 %, 73.2 %, 65.9 %, and 81 %, respectively); however, PPFE-like lesions were present in all patients with PPFE (p = 0.42). Consequently, there was no significant difference in the characteristics of PPFE-like lesions among patients with all forms of IP, except PPFE. PPFE-like lesions were not a significant predictor of prognosis (hazard ratio [HR], 1.16; 95 % confidence interval [CI], 0.64-2.10, p = 0.62); however, patients with PPFE-like lesions under the aortic arch had significantly poorer prognoses (HR, 2.70; 95 % CI, 1.66-4.39, p < 0.001). For craniocaudal extent comparison, patients with IPF with PPFE-like lesions below the level of the carina had significantly poorer prognoses than those without PPFE-like lesions (p = 0.001, overall survival 53.1 and 80.6, respectively).
PPFE-like lesions are common in patients with IP, and their characteristics were not significantly different among all forms of IP, except idiopathic PPFE. The broad extent of PPFE-like lesions is an important predictor of prognosis in patients with IPF.
本研究旨在评估胸膜实质纤维弹性组织增生症（PPFE）样病变在预测慢性间质性肺炎（IP）患者预后中的意义。
本研究纳入了207例行外科肺活检的IP患者。在本研究纳入的患者中，77例患有特发性肺纤维化（IPF），15例患有非特异性间质性肺炎（NSIP），13例患有慢性过敏性肺炎（CHP），41例患有结缔组织病（CTD），3例患有PPFE，58例诊断不明确。对每例IP患者的PPFE样病变的发生率、特征及厚度进行评估。此外，还确定了PPFE样病变对预后的影响。
207例患者中，160例（77.3%）表现出PPFE样病变。PPFE样病变在IPF、NSIP、CHP、CTD及诊断不明确的患者中的发生率相似（分别为79.5%、79.5%、73.2%、65.9%和81%）；然而，所有PPFE患者均存在PPFE样病变（p = 0.42）。因此，除PPFE外，所有形式IP患者的PPFE样病变特征无显著差异。PPFE样病变不是预后的显著预测因素（风险比[HR]为1.16；95%置信区间[CI]为0.64 - 2.10，p = 0.62）；然而，主动脉弓以下有PPFE样病变的患者预后明显较差（HR为2.70；95%CI为1.66 - 4.39，p < 0.001）。在头尾范围比较中，隆突水平以下有PPFE样病变的IPF患者的预后明显比无PPFE样病变的患者差（p = 0.001，总生存率分别为53.1和80.6）。
PPFE样病变在IP患者中很常见，除特发性PPFE外，其特征在所有形式的IP中无显著差异。PPFE样病变的广泛范围是IPF患者预后的重要预测因素。
