Systemic sclerosis-associated interstitial lung disease in the EUSTAR database: analysis by region
Lara D. Veeken, vol. 62, pp. 2178–2188
Abstract
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.
Authors 96
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Alain Lescoat corresponding
Inserm · École des Hautes Études en Santé Publique · Institut de Recherche en Santé, Environnement et Travail · Centre Hospitalier Universitaire de Rennes · Université Rennes 1 · Université de Rennes
Affiliation as printed
CHU Rennes, Inserm, EHESP, Irset (Institut de Recherche en Santé, Environnement et Travail) - UMR_S 1085, Univ Rennes , Rennes, France
Department of Internal Medicine and Clinical Immunology, CHU Rennes, University of Rennes 1 , Rennes, France
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Charité - Universitätsmedizin Berlin
Affiliation as printed
Institute of Biostatistics and Clinical Epidemiology, Charité Universitätsmedizin , Berlin, Germany
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Boehringer Ingelheim (Germany)
Affiliation as printed
Boehringer Ingelheim International GmbH , Ingelheim am Rhein, Germany
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Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia
Affiliation as printed
Rheumatology and Clinical Immunology Unit, ASST Spedali Civili of Brescia , Brescia, Italy
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Leiden University Medical Center
Affiliation as printed
Department of Rheumatology, Leiden University Medical Centre (LUMC) , Leiden, Netherlands
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University Hospital Schleswig-Holstein · University of Lübeck
Affiliation as printed
Department of Rheumatology, University Medical Center Schleswig-Holstein, Campus Lübeck , Lübeck, Germany
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Inserm · Université de Lille · Centre Hospitalier Universitaire de Lille · Institute for Translational Research in Inflammation
Affiliation as printed
CHU Lille, Service de Médecine Interne, Centre de Référence des Maladies Auto-immunes et Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), Univ. Lille , Lille, France
INFINITE—Institute for Translational Research in Inflammation, Univ. Lille , Lille, France
Inserm, U1286 , Lille, France
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Affiliation as printed
Rheumatology Unit, Department of Medicine DIMED, University of Padova , Padova, Italy
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Affiliation as printed
Department of Translational and Precision Medicine, Sapienza University of Rome , Rome, Italy
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Affiliation as printed
Department of Dermatology, University of Cologne , Koln, Nordrhein-Westfalen, Germany
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University of Pavia · Policlinico San Matteo Fondazione
Affiliation as printed
Rheumatology Department, Fondazione IRCCS Policlinico San Matteo, University of Pavia , Pavia, Italy
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Affiliation as printed
Dipartimento Di Scienze Cliniche E Molecolari, Clinica Medica, Università Politecnica Delle Marche , Ancona, Italy
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Affiliation as printed
Department of Rheumatology, Oslo University Hospital-Rikshospitalet , Oslo, Norway
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Affiliation as printed
Department of Rheumatology, University Hospital Zurich , Zurich, Switzerland
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Ospedale Policlinico San Martino · University of Genoa
Affiliation as printed
IRCCS Rheumatology Unit, San Martino Polyclinic , Genoa, Italy
Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa , Genoa, Italy
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Hôpital Cochin · Assistance Publique – Hôpitaux de Paris · Université Paris Descartes
Affiliation as printed
Department of Rheumatology, Cochin Hospital, AP-HP, Paris Descartes University , Paris, France
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Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia
Affiliation as printed
Rheumatology and Clinical Immunology Unit, ASST Spedali Civili of Brescia, Brescia, Italy
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University Hospital Schleswig-Holstein · University of Lübeck
Affiliation as printed
Department of Rheumatology, University Medical Center Schleswig-Holstein, Campus Lübeck, Lübeck, Germany
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Affiliation as printed
Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland
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Affiliation as printed
Department of Rheumatology, Oslo University Hospital-Rikshospitalet, Oslo, Norway
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