系统性硬皮病相关间质性肺疾病中的胸膜肺实质纤维弹性化。
Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease.
机构信息
Department of Rheumatology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
Department of Radiology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
出版信息
Turk J Med Sci. 2022 Feb;52(1):83-88. doi: 10.3906/sag-2107-13. Epub 2022 Feb 22.
BACKGROUND
To explore the frequency and clinical associations of radiologic pleuroparenchymal fibroelastosis (PPFE) in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD).
METHODS
In this single-center retrospective study, high resolution computed tomography (HRCT) images of 105 patients with SSc-ILD were examined for the presence of PPFE. Demographic, clinical, laboratory, and pulmonary function test (PFT) data of patients with and without PPFE were compared.
RESULTS
PPFE was detected in 19 (18.1%) patients ('definite PPFE' in 13 and 'consistent with PPFE' in 6 patients). Patients with PPFE had higher age and longer disease duration than PPFE (-) patients (p < 0.05 for both). Radiologic usual interstitial pneumoniae (UIP) pattern was more frequent (26.3% vs. 4.7%, p = 0.01) and median force vital capacity (FVC) was lower in patients with PPFE (64% vs. 82%, p = 0.005). Spontaneous pneumothorax developed in one patient with PPFE. More deaths occured in PPFE (+) group during follow-up (31% vs. 11%, p = 0.04).
背景
探讨系统性硬化症相关间质性肺病（SSc-ILD）患者中放射学胸膜肺实质纤维弹性化（PPFE）的频率和临床相关性。
方法
在这项单中心回顾性研究中，检查了 105 例 SSc-ILD 患者的高分辨率计算机断层扫描（HRCT）图像，以确定是否存在 PPFE。比较了有和没有 PPFE 的患者的人口统计学、临床、实验室和肺功能测试（PFT）数据。
结果
19 例（18.1%）患者检测出 PPFE（13 例为“明确 PPFE”，6 例为“符合 PPFE”）。与 PPFE（-）患者相比，PPFE 患者的年龄更高，疾病持续时间更长（均为 p < 0.05）。放射学寻常型间质性肺炎（UIP）模式更为常见（26.3%比 4.7%，p = 0.01），PPFE 患者的中位用力肺活量（FVC）更低（64%比 82%，p = 0.005）。1 例 PPFE 患者发生自发性气胸。在随访期间，PPFE（+）组的死亡人数更多（31%比 11%，p = 0.04）。
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