Clinical considerations in ILD最新文献

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S123 Pleuroparenchymal fibroelastosis: clinical, functional and morphologic determinants of mortality 胸膜实质纤维弹性增生:死亡率的临床、功能和形态学决定因素

Clinical considerations in ILD

Pub Date : 2021-02-01 DOI: 10.1136/THORAX-2020-BTSABSTRACTS.128

F. Chua, E. Bartlett, J. Barnett, A. Devaraj, E. Renzoni, A. Nicholson, A. Rice, P. Molyneaux, P. George, M. Kokosi, V. Kouranos, T. Maher, A. Wells, S. Desai

Introduction and Objectives Progressive pleuroparenchymal fibroelastosis (PPFE) is associated with a high symptom burden and frequently co-exists with a separate interstitial lung disease. The prognostic impact of such combinations is unclear and the clinical and computed tomographic (CT) determinants of mortality remain poorly characterised. Methods Patients with a diagnosis of PPFE (2004–19) were retrieved from the Royal Brompton Hospital ILD databases. CTs were evaluated for radiologic features, including: 1) the cranio-caudal extent and the severity of PPFE; 2) the hilar position (ratio of the lung apex to the ‘hilar point’ distance/lung apex to the diaphragmatic dome distance); 3) upper lobe volume loss, and 4) presence of co-existent ILD. Results 139 patients (75 [54%] female; median age 63.5, IQR 52–71.5) were evaluated, including 51 (36.7%) with idiopathic PPFE, 41 (29.5%) with concomitant idiopathic UIP, 17 (12.2%) with hypersensitivity pneumonitis and 8 (5.8%) with autoimmunity. Histopathological information was available in 50 (36%) patients, including from 39 surgical biopsies. 51 deaths were recorded among 130 patients with longitudinal data, yielding a median survival of 3 years. The mean severity of PPFE was negatively correlated with hilar position (r = -0.38, P Conclusions Patients with progressive PPFE have a poor outcome, with a median survival that is comparable to IPF. Identifiable and measurable changes in specific clinical, physiologic and radiologic parameters appear to characterise the adverse prognostic profile of these individuals.

进行性胸膜实质纤维弹性增生症(PPFE)与高症状负担相关，经常与单独的间质性肺疾病共存。这些组合对预后的影响尚不清楚，临床和计算机断层扫描(CT)对死亡率的决定因素仍不明确。方法从皇家布朗普顿医院的ILD数据库中检索诊断为PPFE的患者(2004-19)。ct评估影像学特征，包括:1)颅尾部范围和PPFE的严重程度;肺门位置(肺尖与“肺门点”距离/肺尖与膈穹窿距离之比);3)上肺叶体积损失，4)同时存在ILD。结果139例患者中，女性75例(54%);中位年龄63.5岁，IQR 52-71.5)，其中特发性PPFE 51例(36.7%)，特发性UIP 41例(29.5%)，过敏性肺炎17例(12.2%)，自身免疫8例(5.8%)。50例(36%)患者可获得组织病理学信息，包括39例手术活检。在130例患者的纵向数据中记录了51例死亡，平均生存期为3年。PPFE的平均严重程度与肺门位置呈负相关(r = -0.38, P)结论进行性PPFE患者预后较差，中位生存期与IPF相当。特定临床、生理和放射参数的可识别和可测量的变化似乎是这些个体不良预后特征的特征。

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