EMPIRE Registry, Czech Part: Impact of demographics, pulmonary function and HRCT on survival and clinical course in idiopathic pulmonary fibrosis
Language English Country England, Great Britain Media print-electronic
Document type Journal Article, Meta-Analysis
PubMed
28862397
DOI
10.1111/crj.12700
Knihovny.cz E-resources
- Keywords
- IPF registry, idiopathic pulmonary fibrosis, prognostic factors, pulmonary functions,
- MeSH
- Idiopathic Pulmonary Fibrosis diagnosis epidemiology physiopathology MeSH
- Incidence MeSH
- Middle Aged MeSH
- Humans MeSH
- Lung Volume Measurements MeSH
- Survival Rate trends MeSH
- Follow-Up Studies MeSH
- Lung diagnostic imaging physiopathology MeSH
- Tomography, X-Ray Computed methods MeSH
- Prognosis MeSH
- Disease Progression MeSH
- Registries * MeSH
- Retrospective Studies MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Vital Capacity physiology MeSH
- Check Tag
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Meta-Analysis MeSH
- Geographicals
- Czech Republic epidemiology MeSH
INTRODUCTION: 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. METHODS: Data of 514 subjects consecutively entered since 2012 into Czech EMPIRE IPF registry were analyzed. RESULTS: 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 DLCO [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 DLCO of ≥15% at months 12, 18, and 24 influenced the OS significantly. The fast progressors defined by the DLCO 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 DLCO of ≥15% at month 12 were confirmed as factors negatively influencing OS. CONCLUSIONS: DLCO 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 DLCO analysis into clinical trials and routine practice.
Department of Pneumology and Thoracic Surgery Hospital Na Bulovce Prague Czech Republic
Department of Pneumology Faculty of Medicine and Charles University Hradec Králové Czech Republic
Department of Pulmonary Diseases and Tuberculosis Masaryk Hospital Ústí nad Labem Czech Republic
Department of Respiratory Diseases Jihlava Hospital Czech Republic
Department of Respiratory Diseases Nový Jičín Hospital Czech Republic
Department of Respiratory Diseases Pardubice Hospital Czech Republic
Department of Respiratory Diseases Tomáš Baťa Regional Hospital Zlín Czech Republic
Department of Respiratory Diseases Znojmo Hospital Czech Republic
Institute of Biostatistics and Analyses Masaryk University Brno Czech Republic
PneumoAllergolog Department Kromeříž Hospital Czech Republic
Pulmonary Department České Budějovice Hospital Czech Republic
References provided by Crossref.org
Diagnosis, course and management of hypersensitivity pneumonitis
The natural history of progressive fibrosing interstitial lung diseases
