Clinical Characteristics and Survival of Patients with Idiopathic Pulmonary Fibrosis: Analysis of the Serbian Cohort from the EMPIRE Registry
Year: 2025
Type: article
Study design: Observational Study
Abstract: Background/Objectives: Idiopathic pulmonary fibrosis (IPF) registries are established to enhance understanding of its natural history. Methods: 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. Results: 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 (DLCO) was 38%. At initiation of antifibrotic therapy, median FVC was 73.2% (71.5% for deceased, 75.8% for survivors (p = 0.455), and DLCO was 33.8% (19.9% for deceased, and 35.6% for survivors (p = 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 (p = 0.598). Conclusions: 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 DLCO. These findings highlight the importance of earlier diagnosis and treatment before significant physiological decline to improve outcomes.
Source: Diagnostics
Authors: Sanja Dimic-Janjic, Mihailo Stjepanovic, Slobodan Belic, Dragan Vukosavljevic, Ivan Milivojevic, Nikola Trboljevac, Nikola Nikolic, Slavko Stamenic, Maja Stojanovic, Kristina Stosic, Martina Koziar Vasakova, Ruza Stevic, Nikola Colic, Katarina Lukic, Miroslav Ilic, Lidija Isovic, Nikola Maric, Spasoje Popevic, Violeta Vucinic-Mihailović, Svetlana Kasikovic Lecic, Slavica Mojsilovic, Tatjana Pejcic, Dragana Jovanovic, the Serbian EMPIRE Investigators
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $3,137
OpenAlex ID: W4413411637