BMJ open respiratory research · 2025
Baseline characteristics from the EXCITING-ILD registry.
СтатьяМногоцентровое исследованиеНазвание и аннотация: Перевод в очереди
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Buschulte Katharina, Kabitz Hans-Joachim, Hagmeyer Lars, Hammerl Peter, Esselmann Albert, Wiederhold Conrad, Skowasch Dirk, Stolpe Christoph, Joest Marcus, Veitshans Stefan, Höffgen Marc, Maqhuzu Phillen, Schwarzkopf Larissa, Hellmann Andreas, Pfeifer Michael, Behr Juergen, Karpavicius Rainer, Guenther Andreas, Polke Markus, Höger Philipp, Somogyi Vivien, Lederer Christoph, Markart Philipp, Kreuter Michael
Аннотация
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.
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.