A Patient with Idiopathic Pleuroparenchymal Fibroelastosis Showing a Sustained Pulmonary Function due to Treatment with Pirfenidone

作者：Sato Seidai; Hanibuchi Masaki; Takahashi Mikiko; Fukuda Yuh; Morizumi Shun; Toyoda Yuko; Goto Hisatsugu; Nishioka Yasuhiko^*

来源：Internal Medicine, 2016, 55(5): 497-501.

DOI：10.2169/internalmedicine.55.5047

• idiopathic pleuroparenchymal fibroelastotis
• idiopathic interstitial pneumonia
• pirfenidone
• pulmonary function

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.

• 出版日期2016