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在接受吡非尼酮治疗的特发性肺纤维化患者中,高分辨率计算机断层扫描结果的恶化预示着在用力肺活量首次下降后疾病的进展。

Deterioration of high-resolution computed tomography findings predicts disease progression after initial decline in forced vital capacity in idiopathic pulmonary fibrosis patients treated with pirfenidone.

作者信息

Higo Hisao, Miyahara Nobuaki, Taniguchi Akihiko, Senoo Satoru, Itano Junko, Watanabe Hiromi, Oda Naohiro, Kayatani Hiroe, Ichikawa Hirohisa, Shibayama Takuo, Kajimoto Kazuhiro, Tanimoto Yasushi, Kanehiro Arihiko, Maeda Yoshinobu, Kiura Katsuyuki

机构信息

Department of Hematology, Oncology and Respiratory Medicine, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan.

Department of Allergy and Respiratory Medicine, Okayama University Hospital, Okayama, Japan; Department of Medical Technology, Okayama University Graduate School of Health Sciences, Okayama, Japan.

出版信息

Respir Investig. 2020 May;58(3):185-189. doi: 10.1016/j.resinv.2019.12.007. Epub 2020 Feb 23.

Abstract

BACKGROUND

Pirfenidone suppresses the decline of forced vital capacity (FVC) in patients with idiopathic pulmonary fibrosis (IPF). However, IPF progresses in some patients despite treatment. We analyzed patients with meaningful FVC declines during pirfenidone treatment and explored the factors predictive of disease progression after FVC decline.

METHODS

This study was a retrospective, multicenter, observational study conducted by the Okayama Respiratory Disease Study Group. We defined initial decline in %FVC as 5% or greater per 6-month period during pirfenidone treatment. IPF patients who were treated with pirfenidone and experienced an initial decline from December 2008 to September 2017 were enrolled.

RESULTS

We analyzed 21 patients with IPF. After the initial decline, 4 (19.0%) patients showed improvement in disease, 11 (52.4%) showed stable disease, and 6 (28.6%) showed progressive disease. There was no significant correlation between %FVC reduction on initial decline and subsequent %FVC change (p = 0.475). Deterioration of high-resolution computed tomography (HRCT) findings on initial decline was observed significantly more often in the progressive versus improved/stable disease groups (100% vs 20.0%, p = 0.009).

CONCLUSIONS

We revealed that deterioration of HRCT findings may predict disease progression after the initial decline in %FVC in IPF patients treated with pirfenidone.

摘要

背景

吡非尼酮可抑制特发性肺纤维化(IPF)患者的用力肺活量(FVC)下降。然而,尽管进行了治疗,仍有一些患者的IPF病情进展。我们分析了在吡非尼酮治疗期间FVC有显著下降的患者,并探讨了FVC下降后疾病进展的预测因素。

方法

本研究是由冈山呼吸系统疾病研究组进行的一项回顾性、多中心观察性研究。我们将FVC百分比的初始下降定义为在吡非尼酮治疗期间每6个月下降5%或更多。纳入2008年12月至2017年9月期间接受吡非尼酮治疗且经历了初始下降的IPF患者。

结果

我们分析了21例IPF患者。在初始下降后,4例(19.0%)患者病情改善,11例(52.4%)病情稳定,6例(28.6%)病情进展。初始下降时FVC百分比降低与随后FVC百分比变化之间无显著相关性(p = 0.475)。与病情改善/稳定组相比,病情进展组在初始下降时高分辨率计算机断层扫描(HRCT)结果恶化的情况明显更常见(100%对20.0%,p = 0.009)。

结论

我们发现,HRCT结果恶化可能预测接受吡非尼酮治疗的IPF患者在FVC百分比初始下降后的疾病进展。

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