Takahashi, Mikiko Nippon Medical School
Fukuda, Yuh Nippon Medical School|Itabashi Chuo Medical Center
Morizumi, Shun Tokushima University
The patient was a 68-year-old man presenting with body weight loss and exertional dyspnea. Highresolution 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 inhibitedduring treatment. This is the first case report suggesting the efficacy of pirfenidone for patients with IPPFE.
The Japanese Society of Internal Medicine
Ⓒ 2016 The Japanese Society of Internal Medicine
internalmedicine_55_5_497.pdf 1.63 MB