Okuda Ryo, Hagiwara Eri, Katano Takuma, Ikeda Satoshi, Sekine Akimasa, Kitamura Hideya, Baba Tomohisa, Okudela Koji, Ohashi Kenichi, Ogura Takashi
Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center.
Department of Pathology, Yokohama City University Graduate School of Medicine.
Sarcoidosis Vasc Diffuse Lung Dis. 2019;36(1):39-46. doi: 10.36141/svdld.v36i1.7117. Epub 2019 May 1.
In 2016, the diagnostic criteria for the acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) were revised. However, there have been published few clinical reports on AE-IPF published using the new criteria. The aim of this study was to investigate the incidence of, risk factors for, and mortality due to newly defined AE. Moreover, differences between triggered AE and idiopathic AE were investigated.
The retrospective study was conducted including all IPF patients diagnosed with surgically-proven usual interstitial pneumonia through multi-disciplinary discussion between January 2006 and December 2015. Data were retrieved from a clinical chart review.
A total of 107 patients with newly diagnosed 107 IPF patients were included. The cumulative incidence of initial AE were 9.6% at 1 year, 16.8% at 2 years, 23.9% at 3 years, and 37.3% at 4 years after diagnosis. Three risk factors for AE-IPF development were identified: 1) the minimum peripheral ozygen saturation level of ≤88% during the 6-minute walk test at the time of diagnosis; 2) forced vital capacity (FVC) decreasing by ≥10% in 1 year; and 3) diffusion capacity of the lungs for carbon monoxide (DLco) decreasing by ≥15% in 1 year. There were no significant differences in background (excluding C-reactive protein), survival and treatment between patients with triggered AE and those with idiopathic AE.
The 6-minute walk test and an annual decline in FVC and DLco were predictive factors for AE incidence. The causes of AE-IPF did not affect the prognosis or treatment options in clinical practice.
2016年，特发性肺纤维化（IPF）急性加重（AE）的诊断标准进行了修订。然而，使用新标准发表的关于AE-IPF的临床报告很少。本研究的目的是调查新定义的AE的发病率、危险因素和死亡率。此外，还研究了触发型AE和特发性AE之间的差异。
本回顾性研究纳入了2006年1月至2015年12月期间通过多学科讨论诊断为手术证实的普通型间质性肺炎的所有IPF患者。数据通过临床病历回顾获取。
共纳入107例新诊断的IPF患者。诊断后1年初始AE的累积发病率为9.6%，2年为16.8%，3年为23.9%，4年为37.3%。确定了AE-IPF发生的三个危险因素：1）诊断时6分钟步行试验期间最低外周血氧饱和度水平≤88%；2）1年内用力肺活量（FVC）下降≥10%；3）1年内肺一氧化碳弥散量（DLco）下降≥15%。触发型AE患者和特发性AE患者在背景（不包括C反应蛋白）、生存率和治疗方面无显著差异。
6分钟步行试验以及FVC和DLco的年度下降是AE发病率的预测因素。在临床实践中，AE-IPF的病因不影响预后或治疗选择。
