Systemic sclerosis-associated interstitial lung disease in the EUSTAR database: analysis by region
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Systemic sclerosis-associated interstitial lung disease in the EUSTAR database: analysis by region. / Lescoat, Alain; Huscher, Dörte; Schoof, Nils; Airò, Paolo; de Vries-Bouwstra, Jeska; Riemekasten, Gabriela; Hachulla, Eric; Doria, Andrea; Rosato, Edoardo; Hunzelmann, Nicolas; Montecucco, Carlomaurizio; Gabrielli, Armando; Hoffmann-Vold, Anna-Maria; Distler, Oliver; Ben Shimol, Jennifer; Cutolo, Maurizio; Allanore, Yannick; EUSTAR Collaborators.
in: RHEUMATOLOGY, Jahrgang 62, Nr. 6, 01.06.2023, S. 2178-2188.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Systemic sclerosis-associated interstitial lung disease in the EUSTAR database: analysis by region
AU - Lescoat, Alain
AU - Huscher, Dörte
AU - Schoof, Nils
AU - Airò, Paolo
AU - de Vries-Bouwstra, Jeska
AU - Riemekasten, Gabriela
AU - Hachulla, Eric
AU - Doria, Andrea
AU - Rosato, Edoardo
AU - Hunzelmann, Nicolas
AU - Montecucco, Carlomaurizio
AU - Gabrielli, Armando
AU - Hoffmann-Vold, Anna-Maria
AU - Distler, Oliver
AU - Ben Shimol, Jennifer
AU - Cutolo, Maurizio
AU - Allanore, Yannick
AU - EUSTAR Collaborators
AU - Kötter, Ina
N1 - © The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.
PY - 2023/6/1
Y1 - 2023/6/1
N2 - OBJECTIVES: The prevalence and characteristics of SSc-associated interstitial lung disease (SSc-ILD) vary between geographical regions worldwide. The objectives of this study were to explore the differences in terms of prevalence, phenotype, treatment and prognosis in patients with SSc-ILD from predetermined geographical regions in the EUSTAR database.MATERIAL AND METHODS: Patients were clustered into seven geographical regions. Clinical characteristics and survival of patients with SSc-ILD were compared among these pre-determined regions.RESULTS: For baseline analyses, 9260 SSc patients were included, with 6732 for survival analyses. The prevalence of SSc-ILD in the overall population was 50.2%, ranging from 44.0% in 'Western Europe and Nordic countries' to 67.5% in 'Eastern European, Russia and Baltic countries'. In all regions, anti-topoisomerase antibodies were associated with SSc-ILD. Management also significantly differed; mycophenolate mofetil was prescribed at baseline in 31.6% of patients with SSc-ILD in 'America (North and South)' and 31.7% in 'Middle East' but only 4.3% in 'Asia and Oceania' (P <0.0001). Patients from 'America (North and South)' and 'Middle East' had the highest survival rate at the end of follow-up (85.8% and 85.2%, respectively).CONCLUSIONS: Our study highlights key differences among regions in terms of clinical presentation and prognosis of SSc-ILD. This work also demonstrates that the management of SSc-ILD is highly variable among the different regions considered, suggesting that efforts are still needed for the standardization of medical practice in the treatment of this disease.
AB - OBJECTIVES: The prevalence and characteristics of SSc-associated interstitial lung disease (SSc-ILD) vary between geographical regions worldwide. The objectives of this study were to explore the differences in terms of prevalence, phenotype, treatment and prognosis in patients with SSc-ILD from predetermined geographical regions in the EUSTAR database.MATERIAL AND METHODS: Patients were clustered into seven geographical regions. Clinical characteristics and survival of patients with SSc-ILD were compared among these pre-determined regions.RESULTS: For baseline analyses, 9260 SSc patients were included, with 6732 for survival analyses. The prevalence of SSc-ILD in the overall population was 50.2%, ranging from 44.0% in 'Western Europe and Nordic countries' to 67.5% in 'Eastern European, Russia and Baltic countries'. In all regions, anti-topoisomerase antibodies were associated with SSc-ILD. Management also significantly differed; mycophenolate mofetil was prescribed at baseline in 31.6% of patients with SSc-ILD in 'America (North and South)' and 31.7% in 'Middle East' but only 4.3% in 'Asia and Oceania' (P <0.0001). Patients from 'America (North and South)' and 'Middle East' had the highest survival rate at the end of follow-up (85.8% and 85.2%, respectively).CONCLUSIONS: Our study highlights key differences among regions in terms of clinical presentation and prognosis of SSc-ILD. This work also demonstrates that the management of SSc-ILD is highly variable among the different regions considered, suggesting that efforts are still needed for the standardization of medical practice in the treatment of this disease.
U2 - 10.1093/rheumatology/keac576
DO - 10.1093/rheumatology/keac576
M3 - SCORING: Journal article
C2 - 36222557
VL - 62
SP - 2178
EP - 2188
JO - RHEUMATOLOGY
JF - RHEUMATOLOGY
SN - 1462-0324
IS - 6
ER -