Suzuki Atsushi, Kimura Tomoki, Kataoka Kensuke, Matsuda Toshiaki, Yokoyama Toshiki, Mori Yuta, Kondoh Yasuhiro
Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan.
Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan.
Respir Med Case Rep. 2018 Jan 31;23:103-106. doi: 10.1016/j.rmcr.2018.01.004. eCollection 2018.
Acute exacerbation (AE) is a severe and life-threatening complication of idiopathic pulmonary fibrosis (IPF). In 2016, the definition and diagnostic criteria for AE-IPF were updated by an international working group. The new definition includes any acute, clinically significant respiratory deterioration (both idiopathic and triggered events) characterized by evidence of new widespread alveolar abnormality in patients with IPF. There are no currently proven beneficial management strategies for idiopathic and triggered AE-IPF. This is the first report describing AE-IPF triggered by empyema, which was improved by a combination of corticosteroid, systemic antifungal therapy, local antifungal therapy, and additional pharmacological therapies. Future research may reveal optimal strategies for both idiopathic and triggered AE-IPF.
急性加重（AE）是特发性肺纤维化（IPF）的一种严重且危及生命的并发症。2016年，一个国际工作组更新了AE-IPF的定义和诊断标准。新定义包括IPF患者中任何急性、具有临床意义的呼吸功能恶化（包括特发性和诱发事件），其特征为有新的广泛肺泡异常的证据。目前尚无已证实对特发性和诱发的AE-IPF有益的管理策略。这是第一份描述由脓胸引发的AE-IPF的报告，该病例通过皮质类固醇、全身抗真菌治疗、局部抗真菌治疗及其他药物治疗的联合应用而得到改善。未来的研究可能会揭示针对特发性和诱发的AE-IPF的最佳策略。
