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

Autor: Elaine F. Remmers, John Varga, Virginia D. Steen, Heather Gladue, Francesco Boin, Mary Carns, Jessica K. Gordon, Lorinda Chung, Robyn T. Domsic, Dinesh Khanna, Vivien Hsu, Richard M. Silver, Lesley Ann Saketkoo, Fredrick M. Wigley, Elena Schiopu, Pravitt Gourh, Daniel L. Kastner, Chris T. Derk, Victoria K. Shanmugam, Paula S. Ramos, Thomas A. Medsger, Antonia Valenzuela, Marcin Trojanowski, Lindsey A. Criswell, Ami A. Shah, Reem Kais Jan, Maureen D. Mayes, Avram Goldberg, Nadia D. Morgan
Rok vydání: 2017
Předmět:
0301 basic medicine
Male
autoantibodies
systemic sclerosis
Scleroderma Renal Crisis
computer.software_genre
Severity of Illness Index
Scleroderma
Cohort Studies
0302 clinical medicine
Prevalence
Prospective Studies
Prospective cohort study
skin and connective tissue diseases
Lung
African Americans
screening and diagnosis
Database
integumentary system
Chromosome Mapping
Sclerodactyly
General Medicine
3. Good health
Detection
Cohort
Female
medicine.symptom
Cohort study
4.2 Evaluation of markers and technologies
Adult
Clinical Sciences
Autoimmune Disease
03 medical and health sciences
Databases
Rare Diseases
Clinical Research
medicine
Humans
Factual
Retrospective Studies
030203 arthritis & rheumatology
business.industry
Inflammatory and immune system
Systemic
Retrospective cohort study
medicine.disease
United States
Arthritis & Rheumatology
Black or African American
030104 developmental biology
Cross-Sectional Studies
Socioeconomic Factors
business
computer
Rheumatism
Zdroj: Medicine, vol 96, iss 51
Popis: 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).African American SSc patients were enrolled retrospectively and prospectively over a 30-year period (1987-2016), from 18 academic centers throughout the United States. The cross-sectional prevalence of sociodemographic, clinical, and serological features was evaluated. Factors associated with clinically significant manifestations of SSc were assessed using multivariate logistic regression analyses.The study population included a total of 1009 African American SSc patients, comprised of 84% women. In total, 945 (94%) patients met the 2013 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for SSc, with the remaining 64 (6%) meeting the 1980 ACR or CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) criteria. 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 cohorts of predominantly European ancestry. Also, 1 in 10 patients had a severe Medsger cardiac score of 4. Pulmonary fibrosis evident on computed tomography (CT) chest was present in 43% of patients and was significantly associated with anti-topoisomerase I positivity. 38% of patients with CT evidence of pulmonary fibrosis had a severe restrictive ventilator defect, forced vital capacity (FVC) ≤50% predicted. A significant association was noted between longer disease duration and higher odds of pulmonary hypertension, telangiectasia, and calcinosis. The prevalence of potentially fatal scleroderma renal crisis was 7%, 3.5 times higher than the 2% prevalence reported in the European League Against Rheumatism Scleroderma Trials and Research (EUSTAR) cohort.Our study emphasizes the unique and severe disease burden of SSc in African Americans compared to those of European ancestry.
Databáze: OpenAIRE
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