Tomassetti Sara, Ravaglia Claudia, Piciucchi Sara, Ryu Jay, Wells Athol, Donati Luca, Dubini Alessandra, Klersy Catherine, Luzzi Valentina, Gori Leonardo, Rosi Elisabetta, Lavorini Federico, Poletti Venerino
Department of Clinical and Experimental Medicine, Interventional Pulmonology Unit, Careggi University Hospital, Florence, Italy.
Pulmonary Unit, Department of Diseases of the Thorax, GB Morgagni Hospital, Forlì, Italy.
Front Med (Lausanne). 2023 Jun 2;10:1151922. doi: 10.3389/fmed.2023.1151922. eCollection 2023.
Therapies that slow idiopathic pulmonary fibrosis (IPF) progression are now available and recent studies suggest that the use of antifibrotic therapy may reduce IPF mortality.
The aim of the study was to evaluate whether, to what extent, and for which factors the survival of IPF in a real-life setting has changed in the last 15 years.
Historical eye is an observational study of a large cohort of consecutive IPF patients diagnosed and treated in a referral center for ILDs with prospective intention. We recruited all consecutive IPF patients seen at GB Morgagni Hospital, Forlì, Italy between January 2002 and December 2016 (15 years). We used survival analysis methods to describe and model the time to death or lung transplant and Cox regression to model prevalent and incident patient characteristics (time-dependent Cox models were fitted).
The study comprised 634 patients. The year 2012 identifies the time point of mortality shift (HR 0.58, CI 0.46-0.63, < 0.001). In the more recent cohort, more patients had better preserved lung function, underwent cryobiopsy instead of surgery, and were treated with antifibrotics. Highly significant negative prognostic factors were lung cancer (HR 4.46, 95% CI 3.3-6, < 0.001), hospitalizations (HR 8.37, 95% CI 6.5-10.7, < 0.001), and acute exacerbations (HR 8.37, 95% CI 6.52-10.7, < 0.001). The average antifibrotic treatment effect estimated using propensity score matching showed a significant effect in the reduction of all-cause mortality (ATE coeff -0.23, SE 0.04, < 0.001), acute exacerbations (ATE coeff -0.15, SE 0.04, < 0.001), and hospitalizations (ATE coeff -0.15, SE 0.04, < 0.001) but no effect on lung cancer risk (ATE coeff -0.03, SE 0.03, = 0.4).
Antifibrotic drugs significantly impact hospitalizations, acute exacerbations, and IPF survival. After the introduction of cryobiopsy and antifibrotic drugs, the prognosis of IPF patients has significantly improved together with our ability to detect IPF at an earlier stage.
目前已有减缓特发性肺纤维化（IPF）进展的疗法，近期研究表明使用抗纤维化疗法可能降低IPF死亡率。
本研究旨在评估在现实环境中，IPF患者的生存率在过去15年里是否发生了变化、变化程度如何以及受哪些因素影响。
历史队列研究是对一大群在一家ILD转诊中心连续诊断和治疗的IPF患者进行的前瞻性观察研究。我们纳入了2002年1月至2016年12月（15年）期间在意大利弗利GB莫尔加尼医院就诊的所有连续IPF患者。我们使用生存分析方法来描述和模拟死亡或肺移植时间，并使用Cox回归来模拟现患和新发病例的患者特征（拟合时间依赖性Cox模型）。
该研究共纳入634例患者。2012年是死亡率转变的时间点（风险比0.58，置信区间0.46-0.63，P<0.001）。在最近的队列中，更多患者的肺功能得到更好的保留，接受了冷冻活检而非手术，并且接受了抗纤维化治疗。高度显著的不良预后因素是肺癌（风险比4.46，95%置信区间3.3-6，P<0.001）、住院（风险比8.37，95%置信区间6.5-10.7，P<0.001）和急性加重（风险比8.37，95%置信区间6.52-10.7，P<0.001）。使用倾向评分匹配估计的抗纤维化治疗平均效果显示，在降低全因死亡率（平均治疗效果系数-0.23，标准误0.04，表示P<0.001）、急性加重（平均治疗效果系数-0.15，标准误0.04，表示P<0.001）和住院（平均治疗效果系数-0.15，标准误0.04，表示P<0.001）方面有显著效果，但对肺癌风险无影响（平均治疗效果系数-0.03，标准误0.03，表示P=0.4）。
抗纤维化药物对住院、急性加重和IPF患者的生存有显著影响。在引入冷冻活检和抗纤维化药物后，IPF患者的预后显著改善，同时我们在更早阶段检测IPF的能力也有所提高。
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