Journal Article

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

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

2025

Overview

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.

• Publisher: MDPI AG,Multidisciplinary Digital Publishing Institute (MDPI)
• Subject: antifibrotic treatment / Carbon monoxide / Care and treatment / Comorbidity / Development and progression / diffusion capacity for carbon monoxide / Drug therapy / Fibroblasts / forced vital capacity / idiopathic pulmonary fibrosis / Lung diseases / Lung transplants / Mortality / Mutation / Oxygen therapy / Patient outcomes / Patients / Pulmonary fibrosis / Pulmonology / Quality of life / Tomography / usual interstitial pneumonia