Idiopathic pleuroparenchymal fibroelastosis - Description of a novel clinicopathologic entity
Idiopathic pleuroparenchymal fibroelastosis - Description of a novel clinicopathologic entity
复制标题
DOI：
10.1378/chest.126.6.2007
复制
发表时间：
2004-12-01
期刊：
影响因子：
9.6
通讯作者：
Brown, KK
中科院分区：
文献类型：
--
作者：
Frankel, SK;Cool, CD;Brown, KK
1996年至2001年，我们发现了5例独特的特发性胸膜实质性肺病，其临床表现提示慢性特发性间质性肺炎，胸膜和实质影像学明显受累，上叶占优势，手术肺活检结果不符合任何目前定义的间质性肺炎。病理结果包括：（1）脏层胸膜纤维化严重;（2）显著。均匀的胸膜下纤维弹性组织增生症;（3）远离胸膜的实质保留;（4）轻度、斑片状淋巴浆细胞浸润;（5）纤维化前缘存在少量成纤维细胞灶。在这份报告中，我们描述了特发性胸膜实质弹力纤维增生症的临床、影像学、生理学和病理学表现。
Between 1996 and 2001, we identified five cases of a unique idiopathic pleuroparenchymal lung disease characterized by a clinical presentation suggestive of a chronic idiopathic interstitial pneumonia, marked pleural and parenchymal radiographic involvement with an upper lobe predominance, and surgical lung biopsy findings that did not fit with any of the currently defined interstitial pneumonias. The pathologic findings included the following: (1) intense fibrosis of the visceral pleura; (2) prominent. homogenous, subpleural fibroelastosis; (3) sparing of the parenchyma distant from the pleura; (4) mild, patchy lymphoplasmacytic infiltrates; and (5) small numbers of fibroblastic foci present at the leading edge of the fibrosis. In this report, we characterize the clinical, radiographic, physiologic, and pathologic findings of this entity, which we term idiopathic pleuroparenchymal fibroelastosis.