A Patient with Idiopathic Pleuroparenchymal Fibroelastosis Showing a Sustained Pulmonary Function due to Treatment with Pirfenidone
A Patient with Idiopathic Pleuroparenchymal Fibroelastosis Showing a Sustained Pulmonary Function due to Treatment with Pirfenidone
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DOI：
10.2169/internalmedicine.55.5047
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发表时间：
2016-01-01
影响因子：
1.2
通讯作者：
Nishioka, Yasuhiko
中科院分区：
文献类型：
--
作者：
Sato, Seidai;Hanibuchi, Masaki;Nishioka, Yasuhiko
患者为68岁男性，出现体重减轻和劳力性呼吸困难。胸部高分辨率计算机断层扫描显示胸膜下致密实变，伴有牵引性支气管扩张和容量损失，主要发生在双侧心尖和上叶。右上叶标本的组织病理学分析显示了与特发性胸膜实质纤维弹力炎(IPPFE)相一致的组织学类型。介绍了吡非尼酮的治疗方法，并对其潜在益处进行了展望。吡非尼酮的疗效令人满意，治疗期间用力肺活量的下降得到抑制。这是首例表明吡非尼酮对IPPFE患者有效的病例报告。
The patient was a 68-year-old man presenting with body weight loss and exertional dyspnea. High-resolution computed tomography of the chest showed dense subpleural consolidation with traction bronchiectasis and volume loss predominantly in bilateral apical lesions and upper lobes. A histopathological analysis of a specimen of the right upper lobe showed histological patterns which were consistent with idiopathic pleuroparenchymal fibroelastotis (IPPFE). Treatment with pirfenidone was introduced with the expectation of its potential benefit. The effect of pirfenidone was satisfactory, and a decline in forced vital capacity was inhibited during treatment. This is the first case report suggesting the efficacy of pirfenidone for patients with IPPFE.