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Rheumatology (Oxford). 2019 Nov 4. pii: kez486. doi: 10.1093/rheumatology/kez486. [Epub ahead of print]

Racial differences in systemic sclerosis disease presentation: a European Scleroderma Trials and Research group study.

Collaborators (296)

Randone SB, Bannert B, Iannone F, Maurer B, Jordan S, Dobrota R, Becker M, Mihai C, Becvarare R, Tomčík M, Bielecka OK, Gindzienska-Sieskiewicz E, Karaszewska K, Cutolo M, Pizzorni C, Paolino S, Sulli A, Ruaro B, Alessandri E, Riccardi A, Giacco V, Messitini V, Irace R, Kedor C, Casteleyn V, Hilger J, Hoeppner J, Rednic S, Szabo I, Petcu A, Avouac J, Camelia F, Desbas C, Vlachoyiannopoulos P, Montecucco C, Caporali R, Cavagna L, Stork J, Inanc M, Joven BE, Novak S, Anic F, Varju C, Minier T, Chizzolini C, Allai D, Kucharz EJ, Kotulska A, Kopec-Medrek M, Widuchowska M, Dolnicar AS, Coleiro B, Gabrielli A, Manfredi L, Benfaremo D, Ferrarini A, Bancel DF, Hij A, Lansiaux P, Lazzaroni MG, Hesselstrand R, Wuttge D, Andréasson R, Martinovic D, Bozic I, Radic M, Braun-Moscovici Y, Monaco AL, Furini F, Hunzelmann N, Moinzadeh P, Pellerito R, Caimmi C, Bertoldo E, Morovic-Vergles J, Culo IM, Pecher AC, Santamaria VO, Heitmann S, Codagnone M, Pflugfelder J, Krasowska D, Michalska-Jakubus M, Seidel M, Hasler P, Kretschmar S, Kohm M, Bajocchi G, Salvador MJ, Silva JAPD, Stamenkovic B, Stankovic A, Selmi CF, Santis M, Ceribelli A, Garzanova L, Koneva O, Starovoytova M, Herrick A, Puppo F, Negrini S, Murdaca G, Engelhart M, Szücs G, Szamosi S, de la Puente C, Grande CS, Villanueva MJG, Midtvedt SØ, Hoffmann-Vold AM, Launay D, Sobanski V, Riccieri V, Vasile M, Ionescu RM, Opris D, Sha A, Woods A, Gheorghiu AM, Bojinca M, Sunderkötter C, Ehrchen J, Ingegnoli F, Mouthon L, Dunogue B, Chaigne B, Legendre P, Cantatore FP, Corrado A, Ullman S, Iversen L, von Mühlen CA, Pozzi MR, Eyerich K, Lauffer F, Wiland P, Szmyrka-Kaczmarek M, Sokolik R, Morgiel E, Madej M, Vanthuyne M, Frédéric H, Alegre-Sancho JJ, Aringer M, Herrmann K, Günther C, Westhovens R, Langhe E, Lenaerts J, Anic B, Baresic M, Mayer M, Üprus M, Otsa K, Yavuz S, Granel B, Radominski SC, de C, Müller S, Azevedo VF, Mendoza F, Busquets J, Popa S, Agachi S, Zenone T, Pileckyte M, Stebbings S, Jordan S, Mathieu A, Vacca A, Sampaio-Barros PD, Stamp L, Solanki K, Silva C, Schollum J, Barns-Graham H, Veale D, O'Rourke M, Loyo E, Tineo C, Paulino G, Mohamed WAAA, Rosato E, Gigante A, Oksel F, Yargucu F, Tanaseanu CM, Popescu M, Dumitrascu A, Tiglea I, Foti R, Visalli E, Benenati A, Amato G, Ancuta C, Villiger P, Adler S, Fröhlich J, Kayser C, Eduardo AL, Fathi N, Alii S, Ahmed M, Hasaneen S, Hakeem EE, de la PG, Lefebvre P, Martin JJG, Sibilia J, Chatelus E, Gottenberg JE, Chifflot H, Litinsky I, Galdo FD, Abignano G, Eng S, Seskute G, Butrimiene I, Rugiene R, Karpec D, Pascal M, Kerzberg E, Bianchi W, Bianchi BV, Bianchi DV, Barcellos Y, Castellví I, Millan M, Limonta M, Rimar D, Rosner I, Slobodin G, Couto M, Spertini F, Ribi C, Buss G, Marcoccia A, Bondanini F, Ciani A, Kahl S, Hsu VM, Martin T, Poindron V, Meghit K, Moiseev S, Novikov P, Chung L, Kolstad K, Stark M, Schmeiser T, Thiele A, Majewski D, Zdrojewski Z, Zaneta S, Wierzba K, Martínez-Barrio J, López-Longo FJ, Bernardino V, Moraes-Fontes MF, Rodrigues AC, Riemekasten G, Sommerlatte S, Jendreck S, Arnold S, Levy Y, Rezus E, Cardoneanu A, Burlui AM, Pamuk ON, Puttini PS, Talotta R, Bongiovanni S, Poormoghim H, Andalib E, Almasi S, Kötter I, Krusche M, Cuomo G, Danzo F, Masini F, Gaches F, Michaud M, Cartos F, Belloli L, Casu C, Sfikakis P, Tektonidou M, Furst D, Feldman GR, Ramazan AM, Nurmambet E, Miroto A, Suta C, Andronache I, Huizinga TWJ, de Vries-Bouwstra J.

Author information

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.

KEYWORDS:

organ manifestations; races; systemic sclerosis

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