Miyamoto Atsushi, Morokawa Nasa, Takahashi Yui, Ogawa Kazumasa, Takeyasu Makiko, Murase Kyoko, Hanada Shigeo, Uruga Hironori, Mochizuki Sayaka, Takaya Hisashi, Kurosaki Atsuko, Kishi Kazuma
Department of Respiratory Medicine, Respiratory Center, Toranomon Hospital, Japan.
Intern Med. 2016;55(6):657-61. doi: 10.2169/internalmedicine.55.5259. Epub 2016 Mar 15.
A man in his mid-60's with idiopathic pulmonary fibrosis and hepatitis B-related liver cirrhosis developed exertional dyspnea and a dry cough lasting for three months. High-resolution computed tomography (HRCT) showed increasing bilateral ground-glass opacity superimposed on the usual interstitial pneumonia pattern. Six months after starting pirfenidone therapy, the partial pressure of arterial oxygen at rest increased from 81 to 101 torr, the predicted forced vital capacity (FVC) value increased from 75% to 94% and the ground-glass opacity on HRCT improved. The FVC value was subsequently maintained near or above baseline for 43 months. We concluded that our patient was a super-responder to pirfenidone therapy.
一名65岁左右的男性，患有特发性肺纤维化和乙型肝炎相关肝硬化，出现劳力性呼吸困难和干咳，持续了三个月。高分辨率计算机断层扫描（HRCT）显示，在普通间质性肺炎模式的基础上，双侧磨玻璃影逐渐增多。开始使用吡非尼酮治疗六个月后，静息时动脉血氧分压从81 torr升至101 torr，预计用力肺活量（FVC）值从75%升至94%，HRCT上的磨玻璃影有所改善。随后，FVC值在43个月内维持在接近或高于基线的水平。我们得出结论，该患者对吡非尼酮治疗反应超预期。
