Clinical Characteristics and Survival of Patients with Idiopathic Pulmonary Fibrosis: Analysis of the Serbian Cohort from the EMPIRE Registry
Status PubMed-not-MEDLINE Jazyk angličtina Země Švýcarsko Médium electronic
Typ dokumentu časopisecké články
PubMed
40941610
PubMed Central
PMC12427864
DOI
10.3390/diagnostics15172121
PII: diagnostics15172121
Knihovny.cz E-zdroje
- Klíčová slova
- antifibrotic treatment, diffusion capacity for carbon monoxide, forced vital capacity, idiopathic pulmonary fibrosis, usual interstitial pneumonia,
- Publikační typ
- časopisecké články MeSH
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.
Center for Radiology University Clinical Center of Serbia 11000 Belgrade Serbia
Clinic for Allergology and Immunology University Clinical Center of Serbia 11000 Belgrade Serbia
Clinic for Pulmonology University Clinical Center of Kragujevac 34000 Kragujevac Serbia
Clinic for Pulmonology University Clinical Center of Nis 18000 Nis Serbia
Clinic for Pulmonology University Clinical Center of Serbia 11000 Belgrade Serbia
Faculty of Medicine University of Belgrade 11000 Belgrade Serbia
Faculty of Medicine University of Nis 18000 Nis Serbia
Faculty of Medicine University of Novi Sad 21000 Novi Sad Serbia
Institute for Pulmonary Diseases of Vojvodina 21000 Novi Sad Serbia
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