High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis.
High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis.
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DOI：
10.1186/1465-9921-12-111
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发表时间：
2011-08-23
影响因子：
5.8
通讯作者：
Poletti V
中科院分区：
文献类型：
--
作者：
Piciucchi S;Tomassetti S;Casoni G;Sverzellati N;Carloni A;Dubini A;Gavelli G;Cavazza A;Chilosi M;Poletti V
特发性胸膜实质纤维弹性病（IPPFE）是一种以胸膜和胸膜下实质纤维化为特征的临床病理实体，主要发生在上肺叶。由于这种疾病极其罕见（迄今为止文献中仅描述了7例），IPPFE的定义不明确的病例可能无法识别。临床病程进展，预后差，目前除肺移植外无其他治疗选择。本报告的目的是描述另外两个病例这种罕见的疾病，回顾CT，临床和组织学特征。
Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently described clinical-pathologic entity characterized by pleural and subpleural parenchymal fibrosis, mainly in the upper lobes. As this disease is extremely rare (only 7 cases have been described in the literature to date) poorly defined cases of IPPFE can go unrecognized. The clinical course of disease is progressive and prognosis is poor, with no therapeutic options other than lung transplantation currently available, yet. The aim of this report is to describe two further cases of this rare disease, reviewing CT, clinical and histological features.
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影响因子：
24.3
作者：
Azoulay, E;Paugam, B;Tazi, A
通讯作者：
Tazi, A
影响因子：
7.5
作者：
Becker, Christian D.;Gil, Joan;Padilla, Maria L.
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Padilla, Maria L.
影响因子：
9.6
作者：
Frankel, SK;Cool, CD;Brown, KK
通讯作者：
Brown, KK
影响因子：
6.4
作者：
Rozin, GF;Gomes, MM;Capelozzi, VL
通讯作者：
Capelozzi, VL