Veronika K Jaeger1, Mohammed Tikly2, Dong Xu3, Elise Siegert4, Eric Hachulla5, Paolo Airò6, Gabriele Valentini7, Marco Matucci Cerinic8, Oliver Distler9, Franco Cozzi10, Patricia Carreira11, Yannick Allanore12, Ulf Müller-Ladner13, Lidia P Ananieva14, Alexandra Balbir-Gurman15, Jörg H W Distler16, Laszlo Czirják17, Mengtao Li3, Jörg Henes18, Sergio A Jimenez19, Vanessa Smith20, Nemanja Damjanov21, Christopher P Denton22, Francesco DelGaldo23, Lesley Ann Saketkoo24, Ulrich A Walker1. 1. Department of Rheumatology, University Hospital Basel, Basel, Switzerland. 2. Division of Rheumatology, Chris Hani Baragwanath Academic Hospital, University of the Witwatersrand, Johannesburg, South Africa. 3. Department of Rheumatology, Peking Union Medical College Hospital, Beijing, China. 4. Department of Rheumatology and Immunology, University Hospital Charité, Berlin, Germany. 5. Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Systémiques et Auto-Immunes Rares du Nord et Nord-Ouest (CERAINO), LIRIC, INSERM, Univ. Lille, CHU Lille, Lille, France. 6. UO Reumatologia ed Immunologia Clinica, Spedali Civili, Brescia, Italy. 7. Rheumatology Department, Second University of Naples, Naples, Italy. 8. Department of Experimental and Clinical Rheumatology, Division of Rheumatology AOUC, University of Florence, Florence, Italy. 9. Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland. 10. Rheumatology Unit, Department of Medicine, University of Padova, Padova, Italy. 11. Servicio de Reumatologia, Hospital Universitario 12 de Octubre, Madrid, Spain. 12. Department of Rheumatology A, Paris Descartes University, Cochin Hospital, Paris, France. 13. Justus-Liebig-University of Giessen, Kerckhoff-Klinik, Germany, Bad Nauheim. 14. VA Nasonova Institute of Rheumatology, Moscow, Russian Federation. 15. B. Shine Rheumatology Institute, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion - Institute of Technology, Haifa, Israel. 16. Department of Internal Medicine 3, University of Erlangen-Nuremberg, Erlangen, Germany. 17. Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary. 18. Department of Internal Medicine II, Eberhard-Karls-University Tübingen, Tübingen, Germany. 19. Scleroderma Centre, Thomas Jefferson University, Philadelphia, PA, USA. 20. Faculty of Internal Medicine, Ghent University, Ghent, Belgium. 21. Institute of Rheumatology, University of Belgrade Medical School, Belgrade, Serbia. 22. Department of Rheumatology, University College London, Royal Free Hospital, London, UK. 23. Leeds Musculoskeletal Biomedical Research Unit (LMBRU), University of Leeds, Leeds, UK. 24. Tulane University Lung Centre, University Medical Centre Scleroderma and Sarcoidosis Patient Care and Research Centre, New Orleans, LA, USA.
Abstract
OBJECTIVES: Racial factors play a significant role in SSc. We evaluated differences in SSc presentations between white patients (WP), Asian patients (AP) and black patients (BP) and analysed the effects of geographical locations. METHODS: SSc characteristics of patients from the EUSTAR cohort were cross-sectionally compared across racial groups using survival and multiple logistic regression analyses. RESULTS: The study included 9162 WP, 341 AP and 181 BP. AP developed the first non-RP feature faster than WP but slower than BP. AP were less frequently anti-centromere (ACA; odds ratio (OR) = 0.4, P < 0.001) and more frequently anti-topoisomerase-I autoantibodies (ATA) positive (OR = 1.2, P = 0.068), while BP were less likely to be ACA and ATA positive than were WP [OR(ACA) = 0.3, P < 0.001; OR(ATA) = 0.5, P = 0.020]. AP had less often (OR = 0.7, P = 0.06) and BP more often (OR = 2.7, P < 0.001) diffuse skin involvement than had WP. AP and BP were more likely to have pulmonary hypertension [OR(AP) = 2.6, P < 0.001; OR(BP) = 2.7, P = 0.03 vs WP] and a reduced forced vital capacity [OR(AP) = 2.5, P < 0.001; OR(BP) = 2.4, P < 0.004] than were WP. AP more often had an impaired diffusing capacity of the lung than had BP and WP [OR(AP vs BP) = 1.9, P = 0.038; OR(AP vs WP) = 2.4, P < 0.001]. After RP onset, AP and BP had a higher hazard to die than had WP [hazard ratio (HR) (AP) = 1.6, P = 0.011; HR(BP) = 2.1, P < 0.001]. CONCLUSION: Compared with WP, and mostly independent of geographical location, AP have a faster and earlier disease onset with high prevalences of ATA, pulmonary hypertension and forced vital capacity impairment and higher mortality. BP had the fastest disease onset, a high prevalence of diffuse skin involvement and nominally the highest mortality.
OBJECTIVES: Racial factors play a significant role in SSc. We evaluated differences in SSc presentations between white patients (WP), Asian patients (AP) and black patients (BP) and analysed the effects of geographical locations. METHODS: SSc characteristics of patients from the EUSTAR cohort were cross-sectionally compared across racial groups using survival and multiple logistic regression analyses. RESULTS: The study included 9162 WP, 341 AP and 181 BP. AP developed the first non-RP feature faster than WP but slower than BP. AP were less frequently anti-centromere (ACA; odds ratio (OR) = 0.4, P < 0.001) and more frequently anti-topoisomerase-I autoantibodies (ATA) positive (OR = 1.2, P = 0.068), while BP were less likely to be ACA and ATA positive than were WP [OR(ACA) = 0.3, P < 0.001; OR(ATA) = 0.5, P = 0.020]. AP had less often (OR = 0.7, P = 0.06) and BP more often (OR = 2.7, P < 0.001) diffuse skin involvement than had WP. AP and BP were more likely to have pulmonary hypertension [OR(AP) = 2.6, P < 0.001; OR(BP) = 2.7, P = 0.03 vs WP] and a reduced forced vital capacity [OR(AP) = 2.5, P < 0.001; OR(BP) = 2.4, P < 0.004] than were WP. AP more often had an impaired diffusing capacity of the lung than had BP and WP [OR(AP vs BP) = 1.9, P = 0.038; OR(AP vs WP) = 2.4, P < 0.001]. After RP onset, AP and BP had a higher hazard to die than had WP [hazard ratio (HR) (AP) = 1.6, P = 0.011; HR(BP) = 2.1, P < 0.001]. CONCLUSION: Compared with WP, and mostly independent of geographical location, AP have a faster and earlier disease onset with high prevalences of ATA, pulmonary hypertension and forced vital capacity impairment and higher mortality. BP had the fastest disease onset, a high prevalence of diffuse skin involvement and nominally the highest mortality.
Authors: Lesley Ann Saketkoo; Tracy Frech; Cecília Varjú; Robyn Domsic; Jessica Farrell; Jessica K Gordon; Carina Mihai; Nora Sandorfi; Lee Shapiro; Janet Poole; Elizabeth R Volkmann; Monika Lammi; Kendra McAnally; Helene Alexanderson; Henrik Pettersson; Faye Hant; Masataka Kuwana; Ami A Shah; Vanessa Smith; Vivien Hsu; Otylia Kowal-Bielecka; Shervin Assassi; Maurizio Cutolo; Cristiane Kayser; Victoria K Shanmugam; Madelon C Vonk; Kim Fligelstone; Nancy Baldwin; Kerri Connolly; Anneliese Ronnow; Beata Toth; Maureen Suave; Sue Farrington; Elana J Bernstein; Leslie J Crofford; László Czirják; Kelly Jensen; Monique Hinchclif; Marie Hudson; Matthew R Lammi; Jennifer Mansour; Nadia D Morgan; Fabian Mendoza; Mandana Nikpour; John Pauling; Gabriela Riemekasten; Anne-Marie Russell; Mary Beth Scholand; Elise Seigart; Tatiana Sofia Rodriguez-Reyna; Laura Hummers; Ulrich Walker; Virginia Steen Journal: Best Pract Res Clin Rheumatol Date: 2021-09-15 Impact factor: 4.991