Pleuroparenchymal Fibroelastosis: Its Clinical Characteristics.
Pleuroparenchymal Fibroelastosis: Its Clinical Characteristics.
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DOI：
10.2174/1573398x0904140129125307
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发表时间：
2013-06
影响因子：
0.2
通讯作者：
Watanabe K
中科院分区：
文献类型：
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作者：
Watanabe K
摘要胸膜实质纤维弹性增生症（PPFE）是一种罕见的肺纤维化，临床上以上肺叶纤维化为主。PPFE是一种缓慢进展的疾病，其首发症状为呼吸困难或干咳。气胸引起的胸痛可能是一些患者的第一症状。PPFE患者身材纤细，胸腔扁平或胸前后尺寸异常狭窄。肺活量、总肺活量和弥漫性肺活量下降是PPFE的呼吸功能特征，与特发性肺纤维化（IPF）相似。PPFE和IPF的临床特征最显著的差异是影像学表现，PPFE的病变以上叶为主，IPF的病变以下叶为主。
Pleuroparenchymal fibroelastosis (PPFE) is a rare pulmonary fibrosis that is clinically characterized by upper-lobe predominant fibrosis. PPFE is a slowly progressive disorder and its first symptom is dyspnea or dry cough. Chest pain because of pneumothorax may be the first symptom in some patients. Patients with PPFE are slender with a flat rib cage or abnormally narrowed anterior–posterior thoracic dimension. Decreases in forced vital capacity, total lung capacity, and diffusing capacity are respiratory-function characteristics of PPFE, similar to those seen in idiopathic pulmonary fibrosis (IPF). The most remarkable difference in clinical features between PPFE and IPF is imaging findings, with upper-lobe-predominant lesions in PPFE and lower-lobe-predominant lesions in IPF.