Usual Interstitial Pneumonia Pattern in the Lower Lung Lobes as a Prognostic Factor in Idiopathic Pleuroparenchymal Fibroelastosis
Usual Interstitial Pneumonia Pattern in the Lower Lung Lobes as a Prognostic Factor in Idiopathic Pleuroparenchymal Fibroelastosis
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DOI：
10.1159/000494061
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发表时间：
2019-01-01
期刊：
影响因子：
3.7
通讯作者：
Takahashi, Kazuhisa
中科院分区：
文献类型：
--
作者：
Kato, Motoyasu;Sasaki, Shinichi;Takahashi, Kazuhisa
背景资料：特发性胸膜实质纤维弹性组织增生症（IPPFE）是一种罕见的间质性肺炎，其特征是上叶和胸膜僵硬，这在胸部高分辨率计算机断层扫描（HRCT）上很明显。然而，IPPFE的预后因素尚未确定。目的：探讨影响IPPFE患者生存率的临床预后因素。方法：在2009年4月至2017年9月期间，我们招募了36例经HRCT临床诊断为IPPFE的患者。这些患者被分为短生存期（IPPFE诊断后12个月内死亡）或长生存期（生存超过12个月）组。我们回顾性分析了其临床特征、血清标志物、肺功能检查结果和HRCT表现。结果：12例患者属于短期生存组，24例患者属于长期生存组。在诊断时，HRCT下叶常见间质性肺炎（UIP）模式共存的发生率在短期生存期明显高于长期生存期。多因素分析显示，HRCT下叶UIP模式是预后不良的唯一独立变量。IPPFE患者诊断后的中位生存时间为24个月。在这些IPPFE患者中，UIP模式的生存时间明显短于无UIP模式的患者。结论：我们的研究结果表明，诊断时下叶的UIP模式是IPPFE的独立预后因素。(C)2018 S. Karger AG，巴塞尔
Background: Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare interstitial pneumonia that is characterized by stiffness in both the upper lobes and pleura, which is evident on high resolution computed tomography (HRCT) of the chest. However, prognostic factors for IPPFE have not been identified yet. Objective: We aimed to investigate the clinical prognostic factors affecting survival in patients with IPPFE. Methods: Between April 2009 and September 2017, we enrolled 36 patients who were clinically diagnosed with IPPFE, using HRCT. These patients were classified as either short survival (dead within 12 months from the diagnosis of IPPFE) or long survival (survived for greater than 12 months) groups. We retrospectively analyzed the clinical characteristics, serum markers, pulmonary function test results, and HRCT findings. Results: Twelve patients were classified into the short survival and 24 were categorized into long survival categories. At the time of diagnosis, the incidence of coexistence of a usual interstitial pneumonia (UIP) pattern in the lower lobes on HRCT in the short survival was significantly higher than that in the long survival. Multivariate analysis revealed that a UIP pattern in the lower lobes on HRCT was the only independent variable for poor prognosis. The median survival time from diagnosis in patients with IPPFE was 24 months. Of these patients with IPPFE, the survival time with a UIP pattern was significantly shorter than in those without a UIP pattern. Conclusion: Our findings suggest that a UIP pattern in the lower lobes at the time of diagnosis was an independent prognostic factor for IPPFE. (C) 2018 S. Karger AG, Basel