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

2022; Oxford University Press; Volume: 62; Issue: 6 Linguagem: Inglês

10.1093/rheumatology/keac576

1462-0332

Alain Lescoat, Dörte Huscher, Nils Schoof, Paolo Airò, Jeska de Vries‐Bouwstra, Gabriela Riemekasten, É. Hachulla, Andrea Doria, Edoardo Rosato, Nicolas Hunzelmann, Carlomaurizio Montecucco, Armando Gabrielli, Anna‐Maria Hoffmann‐Vold, Oliver Distler, Jennifer Ben Shimol, Maurizio Cutolo, Yannick Allanore, Marco Matucci‐Cerinic, Ulrich A. Walker, Florenzo Iannone, Radim Bečvář, Otylia Kowal Bielecka, Carmen Pizzorni, Francesco Ciccia, Elise Siegert, Simona Rednic, Panayiotis G. Vlachoyiannopoulos, Jiří Štork, Murat Inanc, Patricia E. Carreira, Srđan Novak, László Czirják, Michele Iudici, Eugeniusz J. Kucharz, Katja Perdan Pirkmajer, Bernard Coleiro, Gianluca Moroncini, Dominique Farge Bancel, Fabian A. Mendoza, Roger Hesselstrand, Mislav Radić, Alexandra Balbir‐Gurman, Andrea Lo Monaco, Raffaele Pellerito, Alessandro Giollo, Jadranka Morović‐Vergles, Christopher P. Denton, Madelon C Vonk, Nemanja Damjanov, Jörg Henes, Vera Ortiz-Santamaría, Stefan Heitmann, Dorota Krasowska, Paul Hasler, Michaela Köhm, Ivan Foeldvari, Gianluigi Bajocchi, Maria João Salvador, Bojana Stamenković, Carlo Selmi, Mohammed Tikly, Lidia P Ananieva, Ariane L. Herrick, Ulf Müller‐Ladner, Klaus Søndergaard, Francesco Puppo, Merete Engelhart, G Szücs, Carlos de la Puente, Valeria Riccieri, Ruxandra Ionescu, Ami Sha, Ana Maria Gheorghiu, Cord Sunderkötter, Jörg H. W. Distler, Francesca Ingegnoli, Luc Mouthon, Vanessa Smith, Francesco Paolo Cantatore, Susanne Ullman, Carlos Alberto von Mühlen, Maria Rosa Pozzi, Kilian Eyerich, Piotr Wiland, Marie Vanthuyne, Juan José Alegre Sancho, Kristine Herrmann, Ellen De Langhe, Branimir Anić, Marko Barešić, Miroslav Mayer, Maria Üprus, Kati Otsa, Şule Yavuz, B. Granel, Carolina de Souza Müller, Svetlana Agachi, Simon Stebbings, Alessandro Mathieu, Alessandra Vacca, Percival D. Sampaio‐Barros, Lisa K. Stamp, Kamal Solanki, Douglas J. Veale, Esthela Loyo, Carmen Tineo, Sergio Toloza, Mengtao Li, Walid Ahmed Abdel Atty Mohamed, Jacek Olas, Fahrettin Öksel, Figen Yargucu, Cristina-Mihaela Tanaseanu, Rosario Foti, Codrina Ancuța, Daniel E. Furst, Britta Maurer, Jacob M. van Laar, Marzena Olesińska, Cristiane Kayser, Nihal Fathi, Paloma García de la Peña Lefebvre, Jorge Juan González Martín, Patrick Carpentier, B. Imbert, Camille Françès, P. Senet, Jean Sibilia, Ira Litinsky, Jean Sénécal, Martial Koenig, France Joval, Tamara Grodzicky, Francesco Del Galdo, Goda Šeškutė, Lesley Ann Saketkoo, Eduardo Kerzberg, Washington Bianchi, Breno Valdetaro Bianchi, I. Castellví, Jasminka Milas-Ahic, Roberta Višević, Massimiliano Limonta, Doron Rimar, Maura Couto, François Spertini, Antonella Marcoccia, Sarah Kahl, Vivien Hsu, Thierry Martin, Sergey Moiseev, Pavel Novikov, Leland W.K. Chung, Tim Schmeiser, Dominik Majewski, Zbigniew Zdrojewski, Julia Martínez‐Barrio, Dinesh Khanna, Vera Bernardino, Lelita Santo, Yair Levy, Elena Rezuş, Ömer Pamuk, Daniel Brito de Araújo, Piercarlo Sarzi‐Puttini, Marek Brzosko, Hadi Poormoghim, Marta Maman, Ina Kötter, Giovanna Cuomo, F. Gaches, Laura Belloli, Petros P. Sfikakis, Juliana Markus, Daniel Furst, Ana-Maria Ramazan, Marie‐Elise Truchetet, P. Jégo, Lorenzo Dagna, Jacob M. van Laar, Lidia Rudnicka, Susana Oliveira, Fabiola Atzeni, Masataka Kuwana, A. Mékinian, M. Martin, Yoshiya Tanaka, Hidekata Yasuoka, Carmen Pilar Simeón‐Aznar, Tatsuya Atsumi, Magda Pârvu, Inês Cordeiro, Nicoletta Del Papa, Thomas Karonitsch, Anna Bazela-Ostromecka, Enrico Selvi, Yasushi Kawaguchi, T. Soukup, Ignasi Rodríguez‐Pintó, Marija Geroldinger‐Simić, Gerard Espinosa, Karen Voigt, Torsten Kubacki, Olena Garmish, Marta Mosca, Ulrich Gerth, Ludmila Antonenko,

Inflammatory Myopathies and Dermatomyositis

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.