| Causes of Scleroderma (MAIN MENU) |
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| Causes of Scleroderma: Genetics |
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| Ethnicity, Race and Geographical Regions |
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| Race and Ethnicity |
| Major Histocompatibility Complex (MHC) class II alleles, haplotypes, and epitopes which confer susceptibility or protection in the fibrosing autoimmune disease systemic sclerosis (SSc): analyses in 1300 Caucasian, African-American and Hispanic cases and 1000 controls. These data indicate unique and multiple HLA class II effects in SSc, especially on autoantibody markers of different sub-phenotypes. Frank C Arnett. Ann Rheum Dis. 12 July 2009. |
| Systemic sclerosis in Sarawak: a profile of patients treated in the Sarawak General Hospital. This study demonstrates the rarity of systemic sclerosis in a multi-ethnic groups, predominantly Chinese, Sarawak natives and Malays with considerable lag time to diagnosis. Diffuse cutaneous systemic scleroderma was more common with rare pulmonary hypertension and renal involvement. C. L. The Rheumatology International 17 April 2009. |
| Ethnic Disparities Among Patients with Pulmonary Hypertension Associated with Systemic Sclerosis. In this cohort of patients, African Americans were more likely to have dcSSc. Among patients with PH, African Americans presented at a younger age than their Caucasian counterparts. Incidence of diastolic dysfunction was higher in the PH population. J Rheumatol 2007 June;34:1277-82. (Also see: Pulmonary Hypertension) |
| Prevalence of renal involvement in Indian patients with systemic sclerosis. SRC (scleroderma renal crisis) is very rare in Indian patients with SSc. However, non-renal crisis abnormalities appear to be as common in Indian patients as compared to the western literature. PubMed. Indian J Med Sci. 2007 Feb;61(2):91-6. (Also see: Renal Involvement) |
| Pulmonary involvement in systemic sclerosis (SSc): Associations with genetic, serologic, sociodemographic, and behavioral factors. Early pulmonary involvement in SSc appears to be influenced by several factors delineated by ethnicity, including racial, socioeconomic, behavioral, and serologic determinants. PubMed. Arthritis Rheum. 2007 Feb 28;57(2):318-326. (Also see: Pulmonary Involvement) |
| Racial Variation in Clinical and Immunological Manifestations of Systemic Sclerosis. After adjusting for gender, disease classification, and disease duration, whites and blacks with SSc differ in some clinical and immunological manifestations of disease. Whether these differences can be attributed to genetic or environmental factors remains unknown. J Rheumatol 2006 February;33:263-8. |
| Race, Scleroderma, and Survival: Why Is There a Difference? Multiple studies have noted that race/ethnicity influences disease expression and outcome in systemic sclerosis. For example, compared to white Americans, African Americans have earlier disease onset, more frequently have diffuse cutaneous involvement, have increased severity of lung disease, and less frequently have anticentromere antibody positivity, which is a marker of more mild disease. Looking specifically at mortality, Laing, et al found that black women with scleroderma had a worse age-adjusted survival rate than did white women with scleroderma. J Rheumatol. 2005 October;32:1873. Editorial. |
| Racial disparities noted in immune system genes. African Americans were significantly more likely to carry genetic variants known to stimulate the inflammatory response. At the same time, genotypes known to dampen the release of anti-inflammatory proteins were more common among African Americans. Other disorders associated with the inflammatory response include premature labor, transplant rejection and autoimmune disorders such as multiple sclerosis and scleroderma - again, all more common among African Americans. EurekAlert! 12/01/04. |
| Ethnic Differences in Cytotoxic T Lymphocyte Associated Antigen 4 Genotype Associations with Systemic Sclerosis. Our data show that the exon 1 (+49) polymorphism of the CTLA-4 gene is associated with systemic sclerosis in African Americans. J Rheumatol No. 1 Jan. 2004;31:85-7. |
| Geographical Regions |
| Geographical variation of disease manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials and Research (EUSTAR) group database. This analysis suggests that eastern centres care for more severe SSc manifestations in Europe. Large differences in patient referral account for a large local variability of SSc presentations and preclude the identification of genetic or environmental factors. Walker UA. (PubMed) Ann Rheum Dis. 2009 Jun;68(6):856-62. |
| Ethnic Influence on Disease Manifestations and Autoantibodies in Chinese-Descent Patients with Systemic Sclerosis. Patients of Chinese descent have milder SSc disease with less frequent joint and gastrointestinal manifestations, less severe vasculopathy, but increased prevalence of myositis and certain autoantibodies. Research is needed to identify determinants (genetic, environmental, and cultural factors) of the relationship between ethnicity and disease. Andrea H.L. Low. The Journal of Rheumatology April 1, 2009 vol. 36 no. 4 787-793. |
| Scleroderma (systemic sclerosis) among Nigerians. Scleroderma among Nigerians is rare as elsewhere and there are certain common characteristics as seen elsewhere as well as certain differences. Adelowo OO. (PubMed) Clin Rheumatol. 2009 May 13. |
| Ethnicity Influence On Clinical And Immunological Manifestations In Systemic Sclerosis: Analysis Of 1139 Brazilian Patients. Ethnicity was associated with differences in the clinical and immunological presentation in a large national SSc cohort in Brazil. P. D. Sampaio-Barros AB0528 EULAR 2007. |
| Disease Subsets, Antinuclear Antibody Profile, and Clinical Features in 127 French and 247 US Adult Patients with Systemic Sclerosis. There are disease classification and SSc-related serum autoantibody differences between French and American patients with SSc. These differences help to explain variations in clinical features reported from different geographic regions. J Rheumatol 2007 January;34:104–9. (Also see: Antibodies) |
| Clinical Features in Arab and Jewish Patients with Systemic Sclerosis in North Israel. This data could support potential influences of ethnic origin on the pathogenesis of SSc and indicates further extension to large group and genetic studies. A. Balbir-Gurman. FRI0319 EULAR 2006. |
| Epidemiology of systemic sclerosis in northwest Greece 1981 to 2002. The incidence and prevalence of SSc in northwest Greece were found to be lower than those of the USA and Australia, and higher than those of northern European countries and Japan. PubMed. Semin Arthritis Rheum. 2005 Apr;34(5):714-20. |
| Scleroderma in Australian aborigines. Scleroderma (systemic sclerosis) has not been reported before in Australian Aborigines. However, scleroderma does occur in indigenous Australians but further studies are needed to confirm the apparent infrequency of centromere-associated limited scleroderma (which is the commonest form of scleroderma in our Caucasian population). PubMed. Intern Med J. 2005 Jan;35(1):60-2. |
| Profile of Antinuclear Antibodies (ANA) in 153 French Patients with Systemic Sclerosis (SSc). Comparison with a Series of 247 US SSc Patients. ANA profile is very similar between SSc patients from French and US centres with a major difference for anti-RNP polymerase III antibodies. As anti-RNA polymerase III antibodies have been described in SSc patients with extensive cutaneous [3] and a high frequency of renal involvement, clinical and epidemiological studies are in progress to explore this difference. O. C. Meyer. FRI0317 EULAR 2004. (Also see: Antibodies) |
| Prevalence of systemic sclerosis in a French multi-ethnic county. The higher prevalence observed for non-Europeans could support potential influences of ethnic origin on the pathogenesis of SSc. PubMed. Rheumatology (Oxford). 2004 Jun 22. |
| Systemic Sclerosis (SSc) in the Northeastern Thais: Clinical Data in 506 Patients. In Thai patients, diffuse scleroderma (dSSc) was more common than Limited Scleroderma (lSSc) with higher prevalence of internal organ involvement. Cardiopulmonary complication is the main cause of death. S. Suwannaroj. FRI0307 EULAR 2004. |
