Idiopathic pleuroparenchymal fibroelastosis: an unrecognized or misdiagnosed entity?
Idiopathic pleuroparenchymal fibroelastosis: an unrecognized or misdiagnosed entity?
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DOI：
10.1038/modpathol.2008.56
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发表时间：
2008-06-01
期刊：
影响因子：
7.5
通讯作者：
Padilla, Maria L.
中科院分区：
文献类型：
--
作者：
Becker, Christian D.;Gil, Joan;Padilla, Maria L.
特发性胸膜实质纤维弹性增生是一种罕见的最近描述的实体，可能被低估和误诊，因为这种实体的认识尚未普及。我们报告两个病例，显示需要包括这种疾病在鉴别诊断的病人主要是胸膜和胸膜下纤维化过程。这种情况是由于弹性纤维主要在上肺叶增生而导致的胸膜和胸膜下实质纤维化增厚。因此，对这种分布的纤维化患者常规进行弹性纤维染色可能有助于确定诊断并将其与通常的间质性肺炎/特发性肺纤维化区分开来。这些患者可能容易发生继发性自发性气胸和术后持续性支气管胸膜瘘。对新诊断病例的持续研究可能会发现有助于产生病原学假设的共同特征或特征。只有更好地了解这种疾病，我们才能希望在未来能够提供除支持性护理和最终肺移植之外的治疗方法，这是目前唯一可用的治疗选择。
Idiopathic pleuroparenchymal fibroelastosis is a rare recently described entity likely to be under- and misdiagnosed, as awareness of this entity is not yet widespread. We report two cases that show the need to include this disease in the differential diagnosis of patients with predominantly pleural and subpleural fibrotic processes. The condition is a fibrotic thickening of the pleura and subpleural parenchyma due to elastic fiber proliferation predominantly in the upper lobes. Performing elastic fiber stains routinely in patients with fibrosis of this distribution may, therefore, aid in establishing the diagnosis and differentiating it from usual interstitial pneumonia/idiopathic pulmonary fibrosis. These patients may be prone to the development of secondary spontaneous pneumothoraces and persistent postoperative bronchopleural fistulae. Continued study of newly diagnosed cases may uncover shared characteristics or features helpful in generating an etiologic hypothesis. Only with better understanding of this disease can we hope in the future to be able to offer treatments other than supportive care and ultimately lung transplantation, which are the only therapeutic options available today.