Pleuroparenchymal fibroelastosis in association with carcinomas
Pleuroparenchymal fibroelastosis in association with carcinomas
复制标题
DOI：
10.1111/resp.12654
复制
发表时间：
2016-01-01
期刊：
影响因子：
6.9
通讯作者：
Kreuter, Michael
中科院分区：
文献类型：
--
作者：
Baroke, Eva;Heussel, Claus Peter;Kreuter, Michael
PPFE是一种少见的疾病，以上叶胸膜纤维化和实质纤维弹力纤维增生症为特征。其病因被认为是特发性的，尽管可能的致病因素已被描述。PPFE与实体肿瘤的关系尚不清楚，以前也没有被考虑过。我们确定了6名PPFE患者，其中4名患者同时患有恶性肿瘤。这一系列的病例表明，PPFE可能是一种不同因素的影响，而不是一种纯粹的特发性疾病。
PPFE is a rare disease characterized by upper lobe pleural fibrosis and parenchymal fibroelastosis. Its aetiology is considered idiopathic, although possible causative factors have been described. An association of PPFE with solid tumours is unknown and has not been considered previously. We identified six patients with PPFE, four of them with a coexisting malignancy. The case series suggests that PPFE might be an implication of varying factors rather than being an exclusively idiopathic condition.