Idiopathic pleuroparenchymal fibroelastosis: three-dimensional computed tomography assessment of upper-lobe lung volume
Idiopathic pleuroparenchymal fibroelastosis: three-dimensional computed tomography assessment of upper-lobe lung volume
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DOI：
10.1183/13993003.00637-2022
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发表时间：
2022-12-01
影响因子：
24.3
通讯作者：
Suda, Takafumi
中科院分区：
文献类型：
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作者：
Fukada, Atsuki;Suzuki, Yuzo;Suda, Takafumi
背景特发性胸膜实质纤维弹力增生症(IPPFE)是一种罕见的间质性肺疾病，以上叶纤维化为主，累及胸膜和胸膜下肺实质。尽管预后不佳，但迄今为止，对于IPPFE的预后决定因素还没有达成共识。由于肺上叶体积减少是IPPFE的一个显著特征，我们假设双侧上叶肺体积(上叶体积)准确地反映了IPPFE患者的病情严重程度和死亡风险。方法本研究回顾分析了132例IPPFE患者(滨松队列中69例，Seirei队列中63例)和45例对照的两个队列。在诊断IPPFE时用三维CT定量测量各肺叶体积，并用预测用力肺活量进行标准化。结果IPPFE患者标准化的上肺叶体积小于对照组的一半，而下肺叶体积并未减少。标准化上叶体积较低的IPPFE患者的存活率明显低于标准上叶体积较大的患者(中位生存期：6.08年对2.48年，P
Background Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is a rare interstitial lung disease characterised by predominant upper-lobe fibrosis involving the pleura and subpleural lung parenchyma. Despite its poor prognosis, there is no consensus on prognostic determinants of iPPFE to date. Because volume loss in the upper lobe is a distinct feature of iPPFE, we hypothesised that the lung volume of the bilateral upper lobes (upper-lobe volume) accurately indicates disease severity and mortality risk in iPPFE patients.Methods This retrospective study assessed two cohorts of 132 patients with iPPFE (69 in Hamamatsu cohort; 63 in Seirei cohort) and 45 controls. Each lobe volume was quantitatively measured using three-dimensional computed tomography at the time of iPPFE diagnosis and standardised using predicted forced vital capacity.Results The standardised upper-lobe volume in iPPFE patients was less than half that of controls, whereas the lower-lobe volume did not decrease. iPPFE patients with lower standardised upper-lobe volume had significantly shorter survival rates than those with higher volume (median survival: 6.08 versus 2.48 years, p