Summary
Through collaborative efforts, the largest multicenter cohort of over 1000 African American patients with scleroderma was established. Study of this cohort, named the Genome Research in African American Scleroderma Patients (GRASP), has enhanced our knowledge about factors associated with significant manifestations of scleroderma in African Americans.
Why was this study done?
The prevalence and severity of scleroderma is higher among African Americans compared to European Americans, but the factors responsible for these differences are unknown. Death associated with scleroderma is also increased among African Americans and is not readily explained by differences in socioeconomic factors or access to healthy care. We therefore sought to increase our understanding of the presentation and factors contributing to the clinical manifestations of scleroderma in African Americans.
How was this study done?
African American patients who were diagnosed with scleroderma and provided consent for participation, were enrolled from 18 academic centers within the United States, including the Johns Hopkins University Scleroderma Center. Over 1000 study participants were recruited over a 30-year period and detailed socioeconomic, clinical, laboratory, and imaging data were collected. These data were used to determine the prevalence of different disease features and to identify factors associated with severe disease in African Americans with scleroderma.
What were the major findings?
The most prevalent features of scleroderma observed in patients in the GRASP cohort was widespread skin thickening over several regions of the body (diffuse scleroderma) and scarring of the lungs (pulmonary fibrosis). While most patients in this cohort were female (84%), as observed in European cohorts, some crucial differences were observed. For example, African Americans in the GRASP cohort had a younger age at disease onset and the prevalence of patients with the potentially fatal complication of scleroderma renal crisis was 3.5-times higher than observed in European cohorts. Importantly, over 97% of patients in the GRASP cohort had a high school education, 59% had a college or post-graduate education, and 97% had health insurance. It is therefore highly unlikely that these factors contributed to the severe scleroderma observed among African American in GRASP.
What is the impact of this work?
The information provided from this work, has increased our understanding of the various manifestations of scleroderma in our African American patients, including factors associated with the severity of their disease. We hope that studying this cohort will allow us to determine the role of genetic factors in the increased susceptibility and severity of scleroderma in African Americans.
This research was supported by:
This work was supported by the Scleroderma Research Foundation (SRF).
Link to original research article:
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. Morgan ND, Shah AA, Mayes MD, Domsic RT, Medsger TA Jr, Steen VD, Varga J, Carns M, Ramos PS, Silver RM, Schiopu E, Khanna D, Hsu V, Gordon JK, Gladue H, Saketkoo LA, Criswell LA, Derk CT, Trojanowski MA, Shanmugam VK, Chung L, Valenzuela A, Jan R, Goldberg A, Remmers EF, Kastner DL, Wigley FM, Gourh P, Boin F. Medicine (Baltimore). 2017 Dec;96(51):e8980. PMID: 29390428 Free PMC Article
