Pleuroparenchymal fibroelastosis: a pattern of chronic lung injury
Pleuroparenchymal fibroelastosis: a pattern of chronic lung injury
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DOI：
10.1016/j.humpath.2014.10.007
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发表时间：
2015-01-01
期刊：
影响因子：
3.3
通讯作者：
Torrealba, Jose R.
中科院分区：
文献类型：
--
作者：
Rosenbaum, Jason N.;Butt, Yasmeen M.;Torrealba, Jose R.
胸膜实质弹性纤维增生症是一种罕见的疾病，目前被描述为上叶胸膜下和间质增生的主要弹性纤维。病因不明，也没有具体的诊断标准。在此我们报告5例PPFE，1男4女，其中3例在尸检时确诊，1例在肺切除时确诊，1例在外科楔形活检时确诊。这一系列的平均诊断年龄为73岁，肺部症状的持续时间从14个月到至少9年不等。2例患者在出现肺部症状前暴露于特定药物（达托霉素和氨苯砜），1例患者在病程中出现嗜酸性粒细胞性肺炎。4例患者有纤维性间质性肺炎的临床证据。我们在所有5例病例中发现了弥漫性实质性纤维弹性组织增生症累及上下肺叶的证据，这表明该疾病可能比以前报道的更弥漫。PPFE实际上可能代表慢性肺损伤的一种模式，而不是一个特定的实体，可能与各种临床放射学条件有关。基于我们在这一系列研究中的发现和最新发表的文献，我们提出了以下一套PPFE的诊断标准：多叶胸膜下和/或小叶中心纤维性间质性肺炎，其特征是非肺不张肺内弹性纤维广泛（>80%）增生，沿着伴无至轻度慢性炎症，无至罕见肉芽肿。(C)2014 Elsevier Inc. All rights reserved.
Pleuroparenchymal fibroelastosis (PPFE) is a rare condition currently described as an upper lobe subpleural and interstitial proliferation of predominantly elastic fibers. The etiology is unknown, and no specific diagnostic criteria have been reported. Here we report 5 cases of PPFE, 1 man and 4 women, 3 of them diagnosed at the time autopsy, 1 diagnosed in an explanted lung, and 1 diagnosed on a surgical wedge biopsy. The average age of diagnosis among this series is 73 years, and the duration of pulmonary symptoms ranged from 14 months to at least 9 years. Two patients had been exposed to specific medications (daptomycin and dapsone) preceding the development of pulmonary symptoms, and 1 patient developed eosinophilic pneumonia in the course of the disease. Four patients had clinical evidence of fibrous interstitial pneumonia. We found evidence of diffuse parenchymal fibroelastosis involving both upper and lower lobes in all 5 cases, suggesting that the disease may be a more diffuse condition than previously reported. PPFE may actually represent a pattern of chronic lung injury rather than a specific entity and may be seen in association with a variety of clinicoradiologic conditions. Based on our findings in this series and the most recent publications of the subject, we propose the following set of diagnostic criteria for PPFE: multilobar subpleural and/or centrilobular fibrous interstitial pneumonia characterized by an extensive (>80%) proliferation of elastic fibers in nonatelectatic lung, along with absent to mild chronic inflammation, and absent to rare granulomas. (C) 2014 Elsevier Inc. All rights reserved.