Distinct Characteristics of Pleuroparenchymal Fibroelastosis With Usual Interstitial Pneumonia Compared With Idiopathic Pulmonary Fibrosis
Distinct Characteristics of Pleuroparenchymal Fibroelastosis With Usual Interstitial Pneumonia Compared With Idiopathic Pulmonary Fibrosis
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DOI：
10.1378/chest.13-2866
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发表时间：
2014-11-01
期刊：
影响因子：
9.6
通讯作者：
Hasegawa, Yoshinori
中科院分区：
文献类型：
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作者：
Oda, Tsuneyuki;Ogura, Takashi;Hasegawa, Yoshinori
背景：胸膜实质弹力纤维增生症（PPFE）是一种罕见的间质性肺炎，有时与组织学常见的间质性肺炎（UIP）模式共存。本研究旨在描述具有 UIP 模式的 PPFE 与特发性肺纤维化 (IPF) 相比的独特临床特征。 方法：我们对 110 名手术肺活检标本上具有组织学 UIP 模式的连续 IPF 患者的医疗记录进行了回顾性审查。根据高分辨率 CT 扫描，符合 PPFE 诊断放射学标准并具有组织学 UIP 模式的患者被纳入研究。 结果：符合 PPFE 诊断放射学标准的 11 名患者中有 9 人经组织学证实患有具有 UIP 模式的 PPFE。具有 UIP 模式组的 PPFE 显示出显着更高的残余体积（1.8 L vs 1.3 L，P
BACKGROUND: Pleuroparenchymal fibroelastosis (PPFE) is a rare form of interstitial pneumonia and sometimes coexists with a histologic usual interstitial pneumonia (UIP) pattern. This study aimed to describe the distinct clinical features of PPFE with UIP pattern compared with idiopathic pulmonary fibrosis (IPF).METHODS: We conducted a retrospective review of the medical records of 110 consecutive patients with IPF with a histologic UIP pattern on surgical lung biopsy specimen. Patients meeting radiologic criteria for the diagnosis of PPFE based on high-resolution CT scan and with a histologic UIP pattern were included.RESULTS: Nine of eleven patients meeting radiologic criteria for the diagnosis of PPFE were histologically confirmed as having PPFE with UIP pattern. The PPFE with UIP pattern group showed a significantly higher residual volume (1.8 L vs 1.3 L, P