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Buschulte, K.* ; Kabitz, H.J.* ; Hagmeyer, L.* ; Hammerl, P.* ; Esselmann, A.* ; Wiederhold, C.* ; Skowasch, D.* ; Stolpe, C.* ; Joest, M.* ; Veitshans, S.* ; Höffgen, M.* ; Maqhuzu, P.N. ; Schwarzkopf, L. ; Hellmann, A.* ; Pfeifer, M.* ; Behr, J.* ; Karpavicius, R.* ; Guenther, A.* ; Polke, M.* ; Höger, P.* ; Somogyi, V.* ; Lederer, C.* ; Markart, P.* ; Kreuter, M.*

Baseline characteristics from the EXCITING-ILD registry.

BMJ Open Respir. Res. 12:e002902 (2025)
Publ. Version/Full Text Research data DOI PMC
Open Access Gold
Creative Commons Lizenzvertrag
BACKGROUND: Interstitial lung diseases (ILDs) comprise a group of more than 200 different subtypes. They vary widely in terms of incidence, prognosis and treatment, yet real-life data from Germany are sparse. METHODS: The prospective Exploring Clinical and Epidemiological Characteristics of Interstitial Lung Diseases (EXCITING)-ILD registry included patients with all different ILD subtypes from different healthcare settings. Follow-up ranged from 36 months to 5 years. Data were analysed descriptively. Baseline characteristics, diagnostic and treatment information are presented as absolute numbers and percentages. The Wilcoxon signed-rank sum test was used to quantify differences between groups. Line plots and bar plots were used for graphical presentation. RESULTS: A total of 601 patients (60.7% men, mean age 64.3 years) from 32 centres were included in the EXCITING-ILD registry. The most common subtypes were sarcoidosis with 26.6% (n=160) and idiopathic pulmonary fibrosis (IPF) with 25.3% (n=152). Pulmonary hypertension was present in 8.7% of patients (n=52), with high incidences in connective tissue disease-associated ILD (16.3%) and pneumoconiosis (27.3%). The mean forced vital capacity was 76.4% predicted, and the mean DLCO-SB (diffusing capacity for carbon monoxide) was 54.1% predicted. The mean time to diagnosis was 38.8 months (SD 64.4) and was significantly shorter when the diagnosis was made after multidisciplinary discussion (31.6 vs 49.2 months, p<0.001). The frequency of surgical lung biopsies decreased over time in the registry, whereas the proportion of cryobiopsies showed a notable increase. In IPF, the number of patients treated with antifibrotics increased from 35.2% before 2015 to 48.4% in 2019. CONCLUSION: The EXCITING-ILD registry describes the frequency of ILD subtypes, ILD-related impairments, selected comorbidities and diagnostic and treatment patterns in a representative German population.
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Publication type Article: Journal article
Document type Scientific Article
Keywords Idiopathic Pulmonary Fibrosis ; Interstitial Fibrosis ; Sarcoidosis; Idiopathic Pulmonary-fibrosis; Interstitial Lung-diseases; Clinical-practice; Epidemiology; Guidelines; Update
ISSN (print) / ISBN 2052-4439
e-ISSN 2052-4439
Journal BMJ Open Respiratory Research
Quellenangaben Volume: 12, Issue: 1, Pages: , Article Number: e002902 Supplement: ,
Publisher BMJ Publishing Group
Publishing Place London
Institute(s) Institute of Health Economics and Health Care Management (IGM)
Grants InterMune