Frankel Stephen K, Cool Carlyne D, Lynch David A, Brown Kevin K
National Jewish Medical and Research Center, 1400 Jackson Street, Denver, CO 80206, USA.
Chest. 2004 Dec;126(6):2007-13. doi: 10.1378/chest.126.6.2007.
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.
1996年至2001年间，我们确诊了5例独特的特发性胸膜肺实质疾病，其临床表现提示为慢性特发性间质性肺炎，影像学显示胸膜和肺实质有明显累及，以上叶为主，且手术肺活检结果不符合目前定义的任何一种间质性肺炎。病理表现如下：（1）脏层胸膜严重纤维化；（2）显著、均匀的胸膜下纤维弹性组织增生；（3）远离胸膜的实质未受累；（4）轻度、散在的淋巴浆细胞浸润；（5）在纤维化前沿有少量成纤维细胞灶。在本报告中，我们描述了该疾病的临床、影像学、生理学和病理学表现，我们将其称为特发性胸膜肺实质纤维弹性组织增生症。
