Race and Ethnicity
Genetics of systemic sclerosis: recent advances. Systemic sclerosis (SSc) is associated with a strong sex bias of between four and nine affected women for one man. African Americans are more likely to develop it than Caucasians, and experience greater morbidity, reduced survival, earlier age of onset, and worse pulmonary disease. The cause of SSc and the reasons for the ethnic disparities remain elusive. Current Opinion in Rheumatology, November 2015, Volume 27, Issue 6, p 521-529.
Early mortality in a multinational systemic sclerosis (SSc) inception cohort. Early mortality in SSc is substantial and prevalent cohorts underestimate mortality in SSc by failing to capture early deaths, particularly in men and those with diffuse disease. PubMed, Arthritis Rheumatol, 12/28/2016. (Also see Prognosis and Mortality)
Caveolin-1 deficiency predisposes African-Americans to systemic sclerosis-interstitial lung disease (SSc-ILD). African-Americans may be predisposed to SSc-ILD due to low baseline caveolin-1 levels in their monocytes. PubMed, Arthritis Rheumatol, 02/27/2014.
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. Ann Rheum Dis, .
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.The Rheumatology International, .
Genes and antibodies for scleroderma and its various symptoms can vary by geographic regions throughout the world. For example, Chinese systemic scleroderma patients typically have milder disease overall, but are more likely to have myositis (muscle inflammation) and certain autoantibodies than other ethnicities.
Association Study of ITGAM, ITGAX, and CD58 Autoimmune Risk Loci in Systemic Sclerosis (SSc): Results from 2 Large European Caucasian Cohorts. These results obtained through 2 large cohorts of SSc patients of European Caucasian ancestry do not support the implication of ITGAM, ITGAX, and CD58 genes in the genetic susceptibility of SSc, although they were recently identified as autoimmune disease risk genes. Rheumatol, .
Anti-RNA Polymerase III Antibody Prevalence and Associated Clinical Manifestations in a Large Series of French Patients with Systemic Sclerosis (SSc): A Cross-sectional Study. The prevalence of anti-RNA polymerase III antibodies in French patients appeared to be lower than in the United States and similar to that in continental Europe. These antibodies were consistently associated with diffuse cutaneous disease and were the most common immunological marker for renal crisis. Rheum. Nov 16,  (Also see RNA-Polymerases Antibodies)
The prognostic factors of systemic sclerosis for survival among Koreans. We investigated the prognostic factors for survival of SSc among 243 Korean patients. Old age, diffuse cutaneous involvement, anti-Scl-70 antibody, and internal organ involvement are risk factors for mortality. Clin Rheum, .
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. PubMed, Ann Rheum Dis, [2009}.
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. The Journal of Rheumatology, ..
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. PubMed, Clin Rheumatol, .
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