Title :

Systemic sclerosis-associated interstitial lung disease in the EUSTAR database: analysis by region.

Author(s) :

Lescoat, Alain [Auteur]
Institut de recherche en santé, environnement et travail [Irset]
École des Hautes Études en Santé Publique [EHESP] [EHESP]
Huscher, D. [Auteur]
Schoof, N. [Auteur]
Airò, P. [Auteur]
De Vries-Bouwstra, J. [Auteur]
Riemekasten, G. [Auteur]
Hachulla, Eric [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Doria, A. [Auteur]
Rosato, E. [Auteur]
Hunzelmann, N. [Auteur]
Montecucco, C. [Auteur]
Gabrielli, A. [Auteur]
Hoffmann-Vold, A. M. [Auteur]
Distler, O. [Auteur]
Ben Shimol, J. [Auteur]
Cutolo, M. [Auteur]
Allanore, Yannick [Auteur]
Hôpital Cochin [AP-HP]

Journal title :

Rheumatology

Abbreviated title :

Rheumatology (Oxford)

Volume number :

62

Pages :

2178–2188

Publication date :

2022-10-14

ISSN :

1462-0332

English keyword(s) :

interstitial lung disease
lung fibrosis
scleroderma
SSc
autoantibodies

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

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 ...
Show more >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.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

Université de Lille
Inserm
CHU Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Submission date :

2024-01-12T00:27:52Z
2024-03-19T14:34:57Z