Jaeger V.K.Tikly M.Xu D.Siegert E.Hachulla E.Airò P.Valentini G.2024-06-122024-06-1220201462-0324https://doi.org/10.1093/rheumatology/kez486https://hdl.handle.net/20.500.14551/16276Racial 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. © 2019 The Author(s) 2019. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved.en10.1093/rheumatology/kez486info:eu-repo/semantics/openAccessOrgan Manifestations; Races; Systemic SclerosisAutoantibody; Dna Topoisomerase; Autoantibody; Dna Topoisomerase; Adult; Article; Asian; Black Person; Caucasian; Centromere; Clinical Feature; Cohort Analysis; Controlled Study; Disease Course; Female; Forced Vital Capacity; Geographic Distribution; Human; Major Clinical Study; Male; Middle Aged; Mortality; Priority Journal; Pulmonary Hypertension; Race Difference; Systemic Sclerosis; Aged; Asian Continental Ancestry Group; Black Person; Caucasian; Complication; Immunology; Lung; Pathophysiology; Systemic Sclerosis; Adult; African Continental Ancestry Group; Aged; Asian Continental Ancestry Group; Autoantibodies; Dna Topoisomerases, Type I; European Continental Ancestry Group; Female; Humans; Hypertension, Pulmonary; Lung; Male; Middle Aged; Scleroderma, SystemicArticle597168416942-s2.0-8508870810831680161Q1