Systemic sclerosis-associated interstitial lung disease in the EUSTAR database: analysis by region
Authors: 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 ,   the EUSTAR collaborators, Cerinic Marco Matucci , Walker Ulrich , Iannone Florenzo , Becvar Radim , Bielecka Otylia Kowal , Pizzorni Carmen , Ciccia Francesco , Siegert Elise , Rednic Simona , Vlachoyiannopoulos P , Stork Jiri , Inanc Murat , Carreira Patricia E , Novak Srdan , Czirják László , Iudici Michele , Kucharz Eugene J , Perdan-Pirkmajer Katja , Coleiro Bernard , Moroncini Gianluca , Bancel Dominique Farge , Mendoza Fabian A , Hesselstrand Roger , Radic Mislav , Balbir-Gurman Alexandra , Monaco Andrea Lo , Pellerito Raffaele , Giollo Alessandro , Morovic-Vergles Jadranka , Denton Christopher , Vonk Madelon , Damjanov Nemanja , Henes Jörg , Santamaria Vera Ortiz , Heitmann Stefan , Krasowska Dorota , Hasler Paul , Kohm Michaela , Foeldvari Ivan , Bajocchi Gianluigi , Salvador Maria João , Stamenkovic Bojana , Selmi Carlo Francesco , Tikly Mohammed , Ananieva Lidia P , Herrick Ariane , Müller-Ladner Ulf , Søndergaard Klaus , Puppo Francesco , Engelhart Merete , Szücs Gabriela , de la Puente Carlos , Riccieri Valeria , Ionescu Ruxandra Maria , Sha Ami , Gheorghiu Ana Maria , Sunderkötter Cord , Distler Jörg , Ingegnoli Francesca , Mouthon Luc , Smith Vanessa , Cantatore Francesco Paolo , Ullman Susanne , von Mühlen Carlos Alberto , Pozzi Maria Rosa , Eyerich Kilian , Wiland Piotr , Vanthuyne Marie , Alegre-Sancho Juan Jose , Herrmann Kristine , De Langhe Ellen , Anic Branimir , Baresic Marko , Mayer Miroslav , Üprus Maria , Otsa Kati , Yavuz Sule , Granel Brigitte , de Souza Müller Carolina , Agachi Svetlana , Stebbings Simon , Mathieu Alessandro , Vacca Alessandra , Sampaio-Barros Percival D , Stamp Lisa , Solanki Kamal , Veale Douglas , Loyo Esthela , Tineo Carmen , Toloza Sergio , Li Mengtao , Mohamed Walid Ahmed Abdel Atty , Olas Jacek , Oksel Fahrettin , Yargucu Figen , Tanaseanu Cristina-Mihaela , Foti Rosario , Ancuta Codrina , Furst Daniel E , Maurer Britta , van Laar Jacob , Olesinska Marzena , Kayser Cristiane , Fathi Nihal , de la Peña Lefebvre Paloma García , Martin Jorge Juan Gonzalez , Carpentier Patrick , Imbert Bernard , Francès Camille , Senet Patricia , Sibilia Jean , Litinsky Ira , Senécal Jean Luc , Koenig Martial , Joval France , Tamara Grodzicky , Del Galdo Francesco , Seskute Goda , Saketkoo Lesley Ann , Kerzberg Eduardo , Bianchi Washington , Bianchi Breno Valdetaro , Castellví Ivan , Milas-Ahic Jasminka , Visevic Roberta , Limonta Massimiliano , Rimar Doron , Couto Maura , Spertini François , Marcoccia Antonella , Kahl Sarah , Hsu Vivien M , Martin Thierry , Moiseev Sergey , Novikov Pavel , Chung Lorinda S , Schmeiser Tim , Majewski Dominik , Zdrojewski Zbigniew , Martínez-Barrio Julia , Khanna Dinesh , Bernardino Vera , Santo Lelita , Levy Yair , Rezus Elena , Pamuk Omer Nuri , de Araujo Daniel Brito , Puttini Piercarlo Sarzi , Brzosko Marek , Poormoghim Hadi , Maman Marta , Kötter Ina , Cuomo Giovanna , Gaches Francis , Belloli Laura , Sfikakis Petros , Markus Juliana , Furst Daniel , Ramazan Ana-Maria , Truchetet Marie-Elise , Jego Patrick , Dagna Lorenzo , van Laar J M , Rudnicka Lidia , Oliveira Susana , Atzeni Fabiola , Kuwana Masataka , Mekinian Arsene , Martin Mickaël , Tanaka Yoshiya , Yasuoka Hidekata , Simeón-Aznar Carmen-Pilar , Atsumi Tatsuya , Parvu Magda , Cordeiro Ines , Del Papa Nicoletta , Karonitsch Thomas , Bazela-Ostromecka Anna , Selvi Enrico , Kawaguchi Yasushi , Soukup Tomas , Rodriguez-Pinto Ignasi , Geroldinger-Simic Marija , Espinosa Gerard , Voigt Karen , Kubacki Torsten , Garmish Olena , Mosca Marta , Gerth Ulrich , Antonenko Ludmila 
Published: October 2022

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 &lt;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.