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特发性肺纤维化患者的临床特征与生存情况:来自EMPIRE注册研究的塞尔维亚队列分析

Clinical Characteristics and Survival of Patients with Idiopathic Pulmonary Fibrosis: Analysis of the Serbian Cohort from the EMPIRE Registry.

作者信息

Dimic-Janjic Sanja, Stjepanovic Mihailo, Belic Slobodan, Vukosavljevic Dragan, Milivojevic Ivan, Trboljevac Nikola, Nikolic Nikola, Stamenic Slavko, Stojanovic Maja, Stosic Kristina, Vasakova Martina Koziar, Stevic Ruza, Colic Nikola, Lukic Katarina, Ilic Miroslav, Isovic Lidija, Maric Nikola, Popevic Spasoje, Vucinic-Mihailović Violeta, Kasikovic Lecic Svetlana, Mojsilovic Slavica, Pejcic Tatjana, Jovanovic Dragana

机构信息

Faculty of Medicine, University of Belgrade, 11000 Belgrade, Serbia.

Clinic for Pulmonology, University Clinical Center of Serbia, 11000 Belgrade, Serbia.

出版信息

Diagnostics (Basel). 2025 Aug 22;15(17):2121. doi: 10.3390/diagnostics15172121.

Abstract

: Idiopathic pulmonary fibrosis (IPF) registries are established to enhance understanding of its natural history. : Serbia (RS) participated in the EMPIRE (European Multi-Partner IPF Registry) from June 2015 to October 2022, involving four centers. The registry included patients over 18 diagnosed with IPF based on the 2011 international criteria. We aimed to gather key clinical, functional, and survival data, along with treatment information for IPF patients in RS, using a centralized electronic case report for consistency. : 188 RS patients participated (median age at diagnosis 65, 63.8% male, 51% smoking history, 56% radiological usual interstitial pneumonia (UIP) pattern). At the diagnosis, median forced vital capacity (FVC) was 73.7% and diffusion capacity for carbon monoxide (DL) was 38%. At initiation of antifibrotic therapy, median FVC was 73.2% (71.5% for deceased, 75.8% for survivors ( = 0.455), and DL was 33.8% (19.9% for deceased, and 35.6% for survivors ( = 0.046)). The median long-term survival from diagnosis was 29.4 months (95% CI: 22.6-36.2 months), and 9.4 months (95% CI: 5.9-12.9 months) from the initiation of therapy, with no difference in the duration of antifibrotic treatment between survivors and deceased ( = 0.598). : The RS EMPIRE cohort represents a younger, less comorbid population with fewer smokers and more probable UIP, factors linked to a favorable prognosis. Nevertheless, survival was poorer than expected, mainly due to advanced disease severity at the time of antifibrotic initiation, as indicated by lower DL. These findings highlight the importance of earlier diagnosis and treatment before significant physiological decline to improve outcomes.

摘要

建立特发性肺纤维化(IPF)登记处是为了增进对其自然病史的了解。塞尔维亚(RS)于2015年6月至2022年10月参与了EMPIRE(欧洲多伙伴IPF登记处),涉及四个中心。该登记处纳入了根据2011年国际标准诊断为IPF的18岁以上患者。我们旨在通过集中的电子病例报告来收集RS地区IPF患者的关键临床、功能和生存数据以及治疗信息,以确保数据的一致性。188名RS患者参与(诊断时的中位年龄为65岁,男性占63.8%,有吸烟史的占51%,放射学表现为普通型间质性肺炎(UIP)模式的占56%)。诊断时,中位用力肺活量(FVC)为73.7%,一氧化碳弥散量(DL)为38%。在开始抗纤维化治疗时,中位FVC为73.2%(死亡患者为71.5%,存活患者为75.8%(P = 0.455)),DL为33.8%(死亡患者为19.9%,存活患者为35.6%(P = 0.046))。从诊断开始的中位长期生存时间为29.4个月(95%置信区间:22.6 - 36.2个月),从开始治疗起为9.4个月(95%置信区间:5.9 - 12.9个月),存活者和死亡者在抗纤维化治疗持续时间上无差异(P = 0.598)。RS的EMPIRE队列代表了一个更年轻、合并症较少、吸烟者较少且更可能为UIP的人群,这些因素与良好的预后相关。然而,生存情况比预期的要差,主要是因为抗纤维化治疗开始时疾病严重程度较高,如较低的DL所示。这些发现凸显了在生理功能显著下降之前进行早期诊断和治疗以改善预后的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b5fd/12427864/6fbc3f516d22/diagnostics-15-02121-g001.jpg

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