Longitudinal Changes in Clinical Features, Management, and Outcomes of Idiopathic Pulmonary Fibrosis A Nationwide Cohort Study
Longitudinal Changes in Clinical Features, Management, and Outcomes of Idiopathic Pulmonary Fibrosis A Nationwide Cohort Study
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DOI：
10.1513/annalsats.202005-451oc
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发表时间：
2021-05-01
影响因子：
8.3
通讯作者：
Park, Moo Suk
中科院分区：
文献类型：
--
作者：
Moon, Sung Woo;Kim, Song Yee;Park, Moo Suk
理由：近几十年来，特发性肺纤维化（IPF）的诊断和治疗建议发生了变化。在韩国，平均预期寿命提高了，未满足的医疗保健需求减少了，计算机断层扫描检查的数量几乎增加了一倍。韩国间质性肺病研究小组对包括 IPF 在内的特发性间质性肺炎进行了全国范围的队列研究，并建立了 IPF 登记处。 目的：利用研究组收集的研究数据，本研究旨在评估临床特征、诊断、治疗和死亡率的纵向变化，并分析药物使用变化对 IPF 相关死亡率的影响程度。 方法：研究人群包括队列研究中新诊断的 IPF 患者（2002 年 1 月至 9 月） 2008 年，n = 1,839，2008 年组）和前瞻性登记（2012 年 1 月至 2018 年 8 月，n = 1,345，2018 年组）。采用Kaplan-Meier法估计生存曲线，并采用Cox回归模型识别各组与死亡相关的危险因素。结果：2018年组比2008年组更年轻，症状更少，蜂窝状症状更少，接受更多的血清学自身免疫标志物和肺功能检查，氧分压值更高，二氧化碳分压值更低，手术活检确诊的频率更低，生存率更高。该组中类固醇的使用和保守治疗有所减少，而 N-乙酰半胱氨酸的使用有所增加。仅 2018 年组使用了抗纤维化药物。在 2008 年的研究组中，N-乙酰半胱氨酸与较低的死亡率相关，而保守治疗与较高的死亡率相关。在 2018 年的研究组中，抗纤维化药物的使用与较低的死亡率相关，而类固醇的使用与较高的死亡率相关。 2008 年和 2018 年非抗纤维化药物亚组的生存率相似。 结论：本研究分析了 17 年的全国 IPF 队列数据。在临床实践中，更早做出IPF诊断，减少类固醇和免疫抑制剂的使用，并给予抗纤维化药物。几十年来，IPF 患者的生存率有所提高，抗纤维化药物的使用始终与生存率的提高相关。
Rationale: In recent decades, diagnosis and treatment recommendations for idiopathic pulmonary fibrosis (IPF) have changed. In Korea, the average life expectancy has increased, unmet healthcare needs have been reduced, and the number of computed tomographic examinations performed has nearly doubled. The Korean Interstitial Lung Disease Study Group conducted a nationwide cohort study for idiopathic interstitial pneumonia, including IPF, and established a registry for IPF.Objectives: Using study data collected by the study group, this study aimed to evaluate longitudinal changes in clinical features, diagnosis, treatment, and mortality and analyze the extent to which changes in medication usage affected IPF-associated mortality.Methods: The study population included newly diagnosed patients with IPF from a cohort study (January 2002 to September 2008, n = 1,839, 2008 group) and prospective registry (January 2012 to August 2018, n = 1,345, 2018 group). Survival curves were estimated using the Kaplan-Meier method, and Cox regression models were used to identify mortalityassociated risk factors in each group.Results: The 2018 group was younger, had fewer symptoms, had less honeycombing, underwent more serologic autoimmune marker and pulmonary function tests, had higher oxygen partial pressure and lower carbon dioxide partial pressure values, was less frequently diagnosed by surgical biopsy, and had better survival than the 2008 group. Steroid use and conservative care declined, whereas N-acetylcysteine use increased in this group. Antifibrotic agents were used in only the 2018 group. In the 2008 group, N-acetylcysteine was associated with lower mortality, whereas conservative care was associated with higher mortality. In the 2018 group, the use of antifibrotic agents was associated with lower mortality, and steroid use was associated with higher mortality. The survival rates in the 2008 and 2018 non-antifibrotic agent subgroups were similar.Conclusions: This study analyzed national IPF cohort data spanning 17 years. In clinical practice, the IPF diagnosis was made earlier, steroid and immunosuppressive agent use was reduced, and antifibrotic agents were administered. The survival of patients with IPF has improved over the decades, and antifibrotic use was consistently associated with improved survival.