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

Nadia D. Morgan¹, Ami A. Shah¹, Maureen D. Mayes², Robyn T. Domsic³, Thomas A. Medsger Jr.⁴, Virginia D. Steen⁵, John Varga⁶, Mary A. Carns⁷, Paula S. Ramos⁸, Richard M. Silver⁹, Elena Schiopu¹⁰, Dinesh Khanna¹⁰, Vivien Hsu¹¹, Jessica K. Gordon¹², Heather Gladue¹³, Lesley A. Saketkoo¹⁴, Lindsey A. Criswell¹⁵, Chris T. Derk¹⁶, Marcin A. Trojanowski¹⁷, Victoria K. Shanmugam¹⁸, Lorinda Chung¹⁹, Antonia Valenzuela²⁰, Reem Jan²¹, Avram Goldberg²², Elaine F. Remmers²³, Daniel L. Kastner²³, Fredrick M. Wigley²⁴, Pravitt Gourh²⁵ and Francesco Boin²⁶, ¹Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, ²Rheumatology, University of Texas Health Science Center at Houston, Houston, TX, ³Medicine - Rheumatology, University of Pittsburgh, Pittsburgh, PA, ⁴Department of Medicine/Rheumatology, University of Pittsburgh, Pittsburgh, PA, ⁵Division of Rheumatology, Department of Medicine, MedStar Georgetown University Hospital, Washington, DC, ⁶Rheumatology and Dermatology, Northwestern University, Feinberg School of Medicine Scleroderma Program, Chicago, IL, ⁷Northwestern University, Feinberg School of Medicine Scleroderma Program, Chicago, IL, ⁸Division of Rheumatology and Immunology, Medical University of South Carolina, Charleston, SC, ⁹Division of Rheumatology and Immunology, Department of Medicine, Medical University of South Carolina, Charleston, SC, ¹⁰University of Michigan, Ann Arbor, MI, ¹¹Rheumatology, Robert Wood Johnson University Scleroderma Program, New Brunswick, NJ, ¹²Rheumatology, Hospital for Special Surgery, New York, NY, ¹³Rheumatology, Arthritis and Osteoporosis Consultants of the Carolinas, Charlotte, NC, ¹⁴Rheumatology, Tulane University School of Medicine, New Orleans, LA, ¹⁵Medicine/Rheumatology, University of California, San Francisco, San Francisco, CA, ¹⁶Rheumatology, University of Pennsylvania, Philadelphia, PA, ¹⁷Boston University School of Medicine, Boston, MA, ¹⁸Rheumatology, The George Washington University, Washington, DC, ¹⁹Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, CA, ²⁰Stanford University School of Medicine, Stanford, CA, ²¹Medicine, Rheumatology, University of Chicago, Chicago, IL, ²²NYU Langone Medical Center, New York, NY, ²³National Institutes of Health (NIH), National Human Genome Research Institute, Bethesda, MD, ²⁴Rheum Div/Mason F Lord, Johns Hopkins University, Baltimore, MD, ²⁵NIAMS-Rheumatology, National Institutes of Health (NIH), National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, ²⁶Rheumatology, University of California, San Francisco, San Francisco, CA

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: African-Americans and systemic sclerosis

Session Information

Date: Sunday, November 5, 2017

Session Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's – Clinical Aspects and Therapeutics I

Session Type: ACR Concurrent Abstract Session

Session Time: 4:30PM-6:00PM

Background/Purpose:

Racial differences exist in the severity of systemic sclerosis (SSc). To enhance our knowledge about SSc in African Americans, we established a comprehensive clinical database from the largest multicenter cohort of African American SSc patients assembled to date (the Genome Research in African American Scleroderma Patients (GRASP) cohort). We compared the phenotypic manifestations of SSc in the GRASP cohort to that reported in the European League Against Rheumatism Scleroderma Trials and Research (EUSTAR) cohort¹.

Methods:

African American SSc patients were enrolled retrospectively and prospectively over a 30-year period (1987 to 2016), from 18 academic centers throughout the United States. 945 (94%) patients met the 2013 ACR/EULAR classification criteria for SSc, with the remaining 64 (6%) meeting the 1980 ACR or CREST criteria. The cross-sectional prevalence of sociodemographic, clinical and serological features was evaluated using data obtained at the time of study enrollment. Factors associated with clinically significant manifestations of SSc were assessed using multivariate logistic regression analyses.

Results:

The study population included a total of 1009 African American SSc patients comprised of 84% women. While 43% were actively employed, 33% required disability support. The majority (57%) had the more severe diffuse subtype and a young age at symptom onset (39.1±13.7 years), in marked contrast to that reported in the EUSTAR cohort. 11% of patients had a severe Medsger cardiac score² of 4 (Figure 1). Pulmonary fibrosis evident on computed tomography (CT) chest was present in 43% of patients, and was significantly associated with anti-topoisomerase I positivity (Table 1). 38% of patients with CT evidence of pulmonary fibrosis had a severe restrictive ventilator defect with forced vital capacity (FVC) £50% predicted. 16% of patients in the GRASP cohort required oxygen therapy compared to 3% in the EUSTAR cohort. A significant association was noted between longer disease duration and higher odds of pulmonary hypertension (Table 1). The prevalence of potentially fatal scleroderma renal crisis was 7%, 3.5 times higher than that reported in the EUSTAR cohort.

Conclusion:

Our study emphasizes the unique and severe disease burden of SSc in African Americans compared to those of European ancestry.

1. Meier FM, et al. Update on the profile of the EUSTAR cohort: an analysis of the EULAR Scleroderma Trials and Research group database. Ann Rheum Dis. 2012;71:1355-60.

2. Medsger TA, Jr., et al. Assessment of disease severity and prognosis. Clin Exp Rheumatol. 2003;21:S42-6.

Disclosure: N. D. Morgan, None; A. A. Shah, None; M. D. Mayes, None; R. T. Domsic, None; T. A. Medsger Jr., None; V. D. Steen, None; J. Varga, BMS, 2,Pfizer Inc, 2; M. A. Carns, None; P. S. Ramos, None; R. M. Silver, None; E. Schiopu, None; D. Khanna, Actelion, Bayer, BoehringerIngelheim, Chemomab, Corbus, Covis, Cytori,Eicos, EMD Serono, Genentech/Roche, Gilead, GSK, Sanofi-Aventis,UCB Pharma, 5,NIH/NIAMS, NIH/NIAID,Bayer, BMS, Genentech/Roche, Pfizer, 2,Eicos, 4; V. Hsu, None; J. K. Gordon, None; H. Gladue, None; L. A. Saketkoo, None; L. A. Criswell, None; C. T. Derk, None; M. A. Trojanowski, None; V. K. Shanmugam, Multiple, 9; L. Chung, Cytori, Actelion, Reata, 5; A. Valenzuela, None; R. Jan, None; A. Goldberg, None; E. F. Remmers, None; D. L. Kastner, None; F. M. Wigley, None; P. Gourh, None; F. Boin, None.

To cite this abstract in AMA style:

Morgan ND, Shah AA, Mayes MD, Domsic RT, Medsger TA Jr., Steen VD, Varga J, Carns MA, 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. 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 [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/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/. Accessed June 3, 2020.

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