Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes
Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes
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DOI：
10.1183/09031936.00165111
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发表时间：
2012-08-01
影响因子：
24.3
通讯作者：
Nicholson, Andrew G.
中科院分区：
文献类型：
--
作者：
Reddy, Taryn L.;Tominaga, Masaki;Nicholson, Andrew G.
胸膜实质弹力纤维增生症 (PPFE) 是一种罕见疾病，其特征主要是上叶胸膜和下肺实质纤维化，后者位于肺泡内，伴有肺泡壁弹性纤维变性。本研究的目的是回顾符合已发表的影像学和组织学标准的病例，并确定可能提示先前被认为是特发性病症的潜在病因的任何常见临床特征。 在 12 名患者（7 名女性，中位年龄 57 岁）中，出现的症状是呼吸短促（12 名患者中有 11 名）和干咳（12 名患者中有 6 名）。七名患者报告在疾病过程中反复感染。其中五人表现出非特异性自身抗体阳性。两名患者有间质性肺病 (ILD) 家族史。12 名患者中有 6 名存在远离胸膜实质变化的肺部疾病高分辨率计算机断层扫描特征（共存纤维化，n=5；支气管扩张，n=1）。在七名从下肺叶采集组织样本的患者中，四名患者表现出较不严重的 PPFE 变化（其中一名患者具有过敏性肺炎的其他特征），三名患者表现出常见的间质性肺炎。 PPFE 是一种独特的临床病理学实体，临床数据表明与复发性肺部感染有关。遗传和自身免疫机制也可能促成这些变化的发生。 PPFE 也可能比以前报道的更广泛的受累，并与不同类型的 ILD 共存。
Pleuroparenchymal fibroelastosis (PPFE) is a rare condition characterised by predominantly upper lobe pleural and subjacent parenchymal fibrosis, the latter being intra-alveolar with accompanying elastosis of the alveolar walls. The aim of this study was to review cases fulfilling published imaging and histological criteria, and identify any common clinical features that may suggest an underlying aetiology for a condition that has previously been regarded as idiopathic.Of 12 patients (seven females, median age 57 yrs), the presenting symptoms were shortness of breath (11 out of 12 patients) and dry cough (six out of 12 patients). Seven patients reported recurrent infections during the course of their disease. Five demonstrated nonspecific autoantibody positivity. Two patients had a family history of interstitial lung disease (ILD).High-resolution computed tomography features of lung disease remote from the pleuroparenchymal changes were present in six out of 12 patients (coexistent fibrosis, n=5; bronchiectasis, n=1). Of seven patients with tissue sampled from the lower lobes, four patients showed less intense PPFE changes (one with additional features of hypersensitivity pneumonitis) and three showed usual interstitial pneumonia.PPFE is a distinct clinicopathological entity, with clinical data suggesting a link to recurrent pulmonary infection. Genetic and autoimmune mechanisms may also contribute to the development of these changes. PPFE may also present with more diffuse involvement than previously reported, and coexist with different patterns of ILD.