Gudmundsson Eyjolfur, Zhao An, Mogulkoc Nesrin, van Beek Frouke, Goos Tinne, Brereton Christopher J, Veltkamp Marcel, Chapman Robert, van Es Hendrik W, Garthwaite Helen, Gholipour Bahareh, Heightman Melissa, Nair Arjun, Pontoppidan Katarina, Savas Recep, Ahmed Asia, Vermant Marie, Unat Omer, Procter Alex, De Sadeleer Laurens, Denneny Emma, Wallis Timothy, Duncan Mark, Taylor Magali, Verleden Stijn, Janes Sam M, Alexander Daniel C, Wells Athol U, Porter Joanna, Jones Mark G, Stewart Iain, van Moorsel Coline H M, Wuyts Wim, Jacob Joseph
Centre for Medical Image Computing, Department of Computer Science, UCL, London, UK.
Department of Respiratory Medicine, Ege University Hospital, Izmir, Turkey.
ERJ Open Res. 2023 Mar 27;9(2). doi: 10.1183/23120541.00637-2022. eCollection 2023 Mar.
Computer quantification of baseline computed tomography (CT) radiological pleuroparenchymal fibroelastosis (PPFE) associates with mortality in idiopathic pulmonary fibrosis (IPF). We examined mortality associations of longitudinal change in computer-quantified PPFE-like lesions in IPF and fibrotic hypersensitivity pneumonitis (FHP).
Two CT scans 6-36 months apart were retrospectively examined in one IPF (n=414) and one FHP population (n=98). Annualised change in computerised upper-zone pleural surface area comprising radiological PPFE-like lesions (Δ-PPFE) was calculated. Δ-PPFE >1.25% defined progressive PPFE above scan noise. Mixed-effects models evaluated Δ-PPFE against change in visual CT interstitial lung disease (ILD) extent and annualised forced vital capacity (FVC) decline. Multivariable models were adjusted for age, sex, smoking history, baseline emphysema presence, antifibrotic use and diffusion capacity of the lung for carbon monoxide. Mortality analyses further adjusted for baseline presence of clinically important PPFE-like lesions and ILD change.
Δ-PPFE associated weakly with ILD and FVC change. 22-26% of IPF and FHP cohorts demonstrated progressive PPFE-like lesions which independently associated with mortality in the IPF cohort (hazard ratio 1.25, 95% CI 1.16-1.34, p<0.0001) and the FHP cohort (hazard ratio 1.16, 95% CI 1.00-1.35, p=0.045).
Progression of PPFE-like lesions independently associates with mortality in IPF and FHP but does not associate strongly with measures of fibrosis progression.
计算机定量分析基线计算机断层扫描（CT）中的胸膜实质纤维弹性组织增生症（PPFE）与特发性肺纤维化（IPF）的死亡率相关。我们研究了IPF和纤维化性过敏性肺炎（FHP）中计算机定量的PPFE样病变的纵向变化与死亡率的关系。
回顾性分析了一个IPF队列（n = 414）和一个FHP队列（n = 98）中间隔6 - 36个月的两次CT扫描。计算包含放射学PPFE样病变的计算机化上叶胸膜表面积的年化变化（Δ-PPFE）。Δ-PPFE>1.25%定义为高于扫描噪声的进展性PPFE。混合效应模型评估Δ-PPFE与视觉CT间质性肺疾病（ILD）范围变化和年化用力肺活量（FVC）下降的关系。多变量模型针对年龄、性别、吸烟史、基线肺气肿存在情况、抗纤维化药物使用和肺一氧化碳弥散量进行了调整。死亡率分析进一步针对临床上重要的PPFE样病变的基线存在情况和ILD变化进行了调整。
Δ-PPFE与ILD和FVC变化的相关性较弱。22%-26%的IPF和FHP队列显示出进展性PPFE样病变，这与IPF队列（风险比1.25，95%CI 1.16 - 1.34，p<0.0001）和FHP队列（风险比1.16，95%CI 1.00 - 1.35，p = 0.045）的死亡率独立相关。
PPFE样病变的进展与IPF和FHP的死亡率独立相关，但与纤维化进展的指标相关性不强。
