African-Brazilians comprise a group of blacks and “pardos.” As racial differences can be associated with distinct presentations, we evaluated the clinical and serological associations of African-Brazilians with systemic sclerosis (SSc).
Sera from 260 adult SSc patients (203 whites and 57 African-Brazilians) were evaluated. Patients with overlap syndromes were excluded. Clinical and demographic data were obtained from an electronic register database. Laboratory analysis included the following: anti-CENP-A/CENP-B, Scl70, RNA polymerase III, Ku, fibrillarin, Th/To, PM-Scl75, and PM-Scl100 by line immunoassay and anti-nuclear antibodies (ANA) by indirect immunofluorescence (IIF) on HEp-2 cells.
African-Brazilian SSc patients presented shorter disease duration (12.8 ± 6.5 vs. 15.9 ± 8.1 years, p = 0.009), higher frequency of nucleolar ANA pattern (28% vs. 13%, p = 0.008), and lower frequencies of centromeric ANA pattern (14% vs. 29%, p = 0.026) and CENP-B (18% vs. 34%, p = 0.017), as well as an association with severe interstitial lung disease (58% vs. 43%; p = 0.044). Further comparison of ethnic groups according to subsets revealed that diffuse SSc African-Brazilian patients presented higher frequency of pulmonary hypertension (p = 0.017), heart involvement (p = 0.037), nucleolar ANA pattern (p = 0.036), anti-fibrillarin antibodies (p = 0.037), and higher mortality (48% vs. 19%; p = 0.009). A different pattern was observed for the limited subset with solely a lower frequency of esophageal involvement (p = 0.050) and centromeric ANA pattern (p = 0.049). Survival analysis showed that African-Brazilians had a higher mortality, when adjusted for age, gender, and clinical subset (RR 2.06, CI 95% 1.10–3.83, p = 0.023).
African-Brazilians have distinct characteristics according to clinical subset and an overall more severe SSc than whites, similar to the blacks from other countries.
• African-Brazilian SSc patients were associated with severe interstitial lung disease and nucleolar ANA pattern when compared to white SSc patients.
• When disease subsets were considered, African-Brazilian patients with diffuse SSc presented association with pulmonary hypertension, heart involvement, nucleolar ANA pattern, and anti-fibrillarin antibodies.
• White SSc patients were associated with centromeric ANA pattern.
• Survival analysis at 5, 10, 15, and 20 years, adjusted for age, gender, and disease subset, was significantly worse in African-Brazilian SSc patients.
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Nihtyanova SI, Denton CP (2010) Autoantibodies as predictive tools in systemic sclerosis. Nat Rev Rheumatol 6:112–116
Reveille JD, Fischbach M, McNearney T, Friedman AW, Aguilar MB, Lisse J, Fritzler MJ, Ahn C, Arnett FC (2001) Systemic sclerosis in 3 US ethnic groups: a comparison of clinical, sociodemographic, serologic, and immunogenetic determinants. Semin Arthritis Rheum 30:332–346
Walker UA, Tyndall A, Czirják L, Denton C, Farge-Bancel D, Kowal-Bielecka O et al (2007) Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group database. Ann Rheum Dis 66:754–763
Gelber AC, Manno RL, Shah AA, Woods A, Le EN, Boin F et al (2013) Race and association with disease manifestations and mortality in scleroderma: a 20-year experience at the Johns Hopkins scleroderma center and review of the literature. Medicine (Baltimore) 92:191–205
Proudman SM, Huq M, Stevens W, Wilson ME, Sahhar J, Baron M, Hudson M, Pope J, Allanore Y, Distler O, Kowal-Bielecka O, Matucci-Cerinic M, Low AHL, Teng GG, Law WG, Santosa A, Nikpour M, Australian Scleroderma Interest Group (ASIG), Canadian Scleroderma Research Group (CSRG), EULAR Scleroderma Trials and Research group (EUSTAR), Singapore Scleroderma Workgroup (SCORE) (2017) What have multicentre registries across the world taught us about the disease features of systemic sclerosis? J Scleroderma Relat Disord 2:169–182
Steen V, Domsic RT, Lucas M, Fertig N, Medsger TA Jr (2012) A clinical and serologic comparison of African-American and Caucasian patients with systemic sclerosis. Arthritis Rheum 64:2986–2994
Silver R, Bogatkevich G, Tourkina E, Nietert P, Hoffman S (2013) Racial differences between blacks and whites with systemic sclerosis. Curr Opin Rheumatol 24:642–648
Ho KT, Reveille JD (2003) The clinical relevance of autoantibodies in scleroderma. Arthritis Res Ther 5:80–93
Steen VD (2005) Autoantibodies in systemic sclerosis. Semin Arthritis Rheum 35:35–42
Van Den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A et al (2013) 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Arthritis Rheum 65:2737–2747
McNeilage LJ, Youngchaiyud U, Whittingham S (1989) Racial differences in antinuclear antibody patterns and clinical manifestations of scleroderma. Arthritis Rheum 32:54–60
Bunn CC, Denton CP, Shi-Wen X, Knight C, Black CM (1998) Anti-RNA polymerases and other autoantibody specificities in systemic sclerosis. Br J Rheumatol 37:15–20
Kuwana M, Okano Y, Pandey JP, Silver RM, Fertig N, Medsger TA (2005) Enzyme-linked immunosorbent assay for detection of anti-RNA polymerase III antibody: analytical accuracy and clinical associations in systemic sclerosis. Arthritis Rheum 52:2425–2432
Sharif R, Fritzler MJ, Mayes MD, Emilio B, McNearney TA, Draeger H et al (2012) Anti-fibrillarin antibody in African American patients with systemic sclerosis: Immunogenetics, clinical features, and survival analysis. J Rheumatol 38:1622–1630
Rodriguez-Reyna TS, Hinojosa-Azaola A, Martinez-Reyes C, Nuez-Alvarez CA, Torrico-Lavayen R, Garca-Hernndez JL, Cabiedes-Contreras J (2011) Distinctive autoantibody profile in Mexican Mestizo systemic sclerosis patients. Autoimmunity 44:576–584
LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA Jr, Rowell NWF (1988) Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 15:202–205
Gomes RC, Silva MF, Kozu K, Bonfá E, Pereira E, Terreri MT et al (2016) Features of 847 childhood-onset systemic lupus erythematosus patients in three age groups at diagnosis: a Brazilian multicenter study. Arthritis Care Res 68:1736–1741
Furst DE, Clements PJ, Steen VD, Medsger TA Jr, Masi AT, D’Angelo WA et al (1998) The modified Rodnan skin score is an accurate reflection of skin biopsy thickness in systemic sclerosis. J Rheumatol 25:84–88
IBGE (2010) Censo Demográfico 2010. Características da População e dos Domicílios. Inst Bras Geografia Estatística 48
Tishkoff SA, Williams SM (2002) Genetic analysis of African populations: human evolution and complex disease. Nat Rev Genet 3:611–621
Pena SDJ, Di Pietro G, Fuchshuber-Moraes M, Genro JP, Hutz MH, Kehdy FS et al (2011) The genomic ancestry of individuals from different geographical regions of Brazil is more uniform than expected. PLoS One 6:e17063
Horimoto AMC, Matos EN, Costa MR, Takahashi F, Rezende MC, Kanomata LB et al (2017) Incidence and prevalence of systemic sclerosis in Campo Grande, State of Mato Grosso do Sul, Brazil. Rev Bras Reumatol 57:107–114
Müller CDS, Paiva S, Azevedo VF (2011) Autoantibody profile and clinical correlation in a group of patients with systemic sclerosis in southern. Brazil. 51(4):314–324
Kuwana M, Okano Y, Kaburaki J, Tojo T, Medsger TA (1994) Racial differences in the distribution of systemic sclerosis-related serum antinuclear antibodies. Arthritis Rheum 37:902–906
Meyer OC, Fertig N, Lucas M, Somogyi N, Medsger TA (2007) Disease subsets, antinuclear antibody profile, and clinical features in 127 French and 247 US adult patients with systemic sclerosis. J Rheumatol 34:104–109
Meier FMP, Frommer KW, Dinser R, Walker UA, Czirjak L, Denton CP, Allanore Y, Distler O, Riemekasten G, Valentini G, Müller-Ladner U, EUSTAR Co-authors (2012) Update on the profile of the EUSTAR cohort: an analysis of the EULAR Scleroderma Trials and Research group database. Ann Rheum Dis 71:1355–1360
Patterson KA, Roberts-Thomson PJ, Lester S, Tan JA, Hakendorf P, Rischmueller M, Zochling J, Sahhar J, Nash P, Roddy J, Hill C, Nikpour M, Stevens W, Proudman SM, Walker JG (2015) Interpretation of an extended autoantibody profile in a well-characterized Australian systemic sclerosis (scleroderma) cohort using principal components analysis. Arthritis Rheumatol 67:3234–3244
Morgan ND, Shah AA, Mayes MD, Domsic RT, Medsger TA Jr, Steen VD et al (2017) Clinical and serological features of systemic sclerosis in a multicenter African American cohort analysis of the genome research in African American scleroderma patients clinical database. Medicine (Baltimore) 51:e8980
Tager RE, Tikly M (1999) Clinical and laboratory manifestations of systemic sclerosis (scleroderma) in Black South Africans. Rheumatology 38:397–400
Adelowo OO, Oguntona S (2009) Scleroderma (systemic sclerosis) among Nigerians. Clin Rheumatol 28:1121–1125
Krzyscczak M, Li Y, Ross SJ, Ceribelli A, Chan EKL, Bubb MR et al (2011) Gender and ethnicity differences in the prevalence of scleroderma-related antibodies. Clin Rheumatol 30:1333–1339
McNearney TA, Reveille JD, Fischbach M, Friedman AW, Lisse JR, Goel N et al (2007) Pulmonary involvement in systemic sclerosis: associations with genetic, serologic, sociodemographic, and behavioral factors. Arthritis Care Res 57:318–326
Ashmore P, Tikly M, Wong M, Ickinger C (2018) Interstitial lung disease in South Africans with systemic sclerosis. Rheumatol Int 38:657–662
Laing TJ, Gillespie BW, Toth MB, Mayes MD, Gallavan RH Jr, Burns CJ et al (1997) Racial differences in scleroderma among women in Michigan. Arthritis Rheum 40:734–742
Blanco I, Mathai S, Shafiq M, Boyce D, Kolb TM, Chami H et al (2014) Severity of systemic sclerosis-associated pulmonary arterial hypertension in African Americans. Medicine (Baltimore) 93:177–185
Villalba WO, Sampaio-Barros PD, Pereira MC, Cerqueira EMFP, Leme CA, Marques-Neto JF, Paschoal IA (2007) Six-minute walk test for the evaluation of pulmonary disease severity in scleroderma patients. Chest 131:217–222
Villalta D, Imbastaro T, Di Giovanni S, Lauriti C, Gabini M, Turi MC, Bizzaro N (2012) Diagnostic accuracy and predictive value of extended autoantibody profile in systemic sclerosis. Autoimmun Rev 12:114–120
Nandiwada SL, Peterson LK, Mayes MD, Jaskowski TD, Malmberg E, Assassi S, Satoh M, Tebo AE (2016) Ethnic differences in autoantibody diversity and hierarchy: more clues from a US cohort of patients with systemic sclerosis. J Rheumatol 43:1816–1824
Damoiseaux J, Andrade LEC, Carballo OG, Konrad K, Francescantonio PLC, Fritzler MJ et al (2019) Clinical relevance of HEp-2 indirect immunofluorescent patterns: the International Consensus on ANA patterns (ICAP) perspective. Ann Rheum Dis. https://doi.org/10.1136/annrheumdis-2018-214436
This study was approved by the local research ethics committee (no. 1.627.446).
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Mendes, C., Viana, V.S.T., Pasoto, S.G. et al. Clinical and laboratory features of African-Brazilian patients with systemic sclerosis. Clin Rheumatol 39, 9–17 (2020) doi:10.1007/s10067-019-04575-5
- Diffuse scleroderma
- Limited scleroderma
- Systemic sclerosis