Doubková Martina, Švancara Jan, Svoboda Michal, Šterclová Martina, Bartoš Vladimír, Plačková Martina, Lacina Ladislav, Žurková Monika, Binková Ilona, Bittenglová Radka, Lošťáková Vladimíra, Merta Zdeněk, Šišková Lenka, Tyl Richard, Lisá Pavlína, Šuldová Hana, Petřík František, Pšikalová Jana, Řihák Vladimír, Snížek Tomáš, Reiterer Pavel, Homolka Jiří, Musilová Pavlína, Lněnička Jaroslav, Palúch Peter, Hrdina Roman, Králová Renata, Hortvíková Hana, Strenková Jana, Vašáková Martina
Department of Phthisiology Pulmonary Diseases and Tuberculosis, Masaryk University Faculty of Medicine and University Hospital, Brno, Czech Republic.
Institute of Biostatistics and Analyses, Masaryk University, Brno, Czech Republic.
Clin Respir J. 2018 Apr;12(4):1526-1535. doi: 10.1111/crj.12700. Epub 2017 Sep 26.
Prognostic factors of idiopathic pulmonary fibrosis (IPF) currently recognized include changes in vital capacity and radiologic findings. However, most of the prognostic studies in IPF are based on clinical studies with preselected IPF populations. Therefore, we decided to analyze the factors influencing IPF prognosis based on the real-practice data from our IPF registry.
Data of 514 subjects consecutively entered since 2012 into Czech EMPIRE IPF registry were analyzed.
Median age of our patient cohort was 67 years (50-82). Median overall survival (OS) of the cohort was 63.1 months. The clinical course of IPF according to FVC (forced vital capacity) changes was stabilized in 32.8% of patients (29.7% according to DL [diffuse lung capacity] changes), slowly progressive in 39.5% (45%), rapidly progressive in 23.5% (20.7%); and 1.7% patients had at least one acute exacerbation during follow-up. Deterioration in FVC of ≥10% at month 12 and in DL of ≥15% at months 12, 18, and 24 influenced the OS significantly. The fast progressors defined by the DL decline rate had higher risk of death compared to those defined by the FVC change over time. In multivariate analysis, age ≥70 years, interstitial HRCT scores ≥3, and change in DL of ≥15% at month 12 were confirmed as factors negatively influencing OS.
DL changes over time were shown as a better predictor of mortality compared with FVC changes in our study. In our opinion it is necessary to implement the DL analysis into clinical trials and routine practice.
目前公认的特发性肺纤维化（IPF）预后因素包括肺活量变化和影像学表现。然而，大多数IPF预后研究基于对预先选择的IPF人群的临床研究。因此，我们决定基于我们IPF登记处的实际数据来分析影响IPF预后的因素。
分析了自2012年以来连续纳入捷克EMPIRE IPF登记处的514名受试者的数据。
我们患者队列的中位年龄为67岁（50 - 82岁）。该队列的中位总生存期（OS）为63.1个月。根据用力肺活量（FVC）变化，32.8%的患者IPF临床病程稳定（根据肺弥散量[DL]变化为29.7%），39.5%（45%）缓慢进展，23.5%（20.7%）快速进展；1.7%的患者在随访期间至少有一次急性加重。第12个月时FVC下降≥10%以及第12、18和24个月时DL下降≥15%对总生存期有显著影响。与根据FVC随时间变化定义的快速进展者相比，根据DL下降率定义的快速进展者死亡风险更高。在多变量分析中，年龄≥70岁、间质HRCT评分≥3以及第12个月时DL变化≥15%被确认为对总生存期有负面影响的因素。
在我们的研究中，与FVC变化相比，DL随时间的变化被证明是更好的死亡率预测指标。我们认为有必要将DL分析纳入临床试验和常规实践。
