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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 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.
PMID
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Authors

Mayor MeshTerms
Keywords
Journal Title medicine
Publication Year Start




PMID- 29390428
OWN - NLM
STAT- MEDLINE
DCOM- 20180213
LR  - 20180213
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Linking)
VI  - 96
IP  - 51
DP  - 2017 Dec
TI  - 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.
PG  - e8980
LID - 10.1097/MD.0000000000008980 [doi]
AB  - 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.
CI  - Copyright (c) 2017 The Authors. Published by Wolters Kluwer Health, Inc. All
      rights reserved.
FAU - Morgan, Nadia D
AU  - Morgan ND
AD  - Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore,
      MD.
FAU - Shah, Ami A
AU  - Shah AA
AD  - Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore,
      MD.
FAU - Mayes, Maureen D
AU  - Mayes MD
AD  - Division of Rheumatology, University of Texas-McGovern Medical School, Houston,
      TX.
FAU - Domsic, Robyn T
AU  - Domsic RT
AD  - Division of Rheumatology, University of Pittsburgh, PA.
FAU - Medsger, Thomas A Jr
AU  - Medsger TA Jr
AD  - Division of Rheumatology, University of Pittsburgh, PA.
FAU - Steen, Virginia D
AU  - Steen VD
AD  - Division of Rheumatology, Georgetown University School of Medicine, Washington,
      DC.
FAU - Varga, John
AU  - Varga J
AD  - Division of Rheumatology, Northwestern University, Feinberg School of Medicine,
      Chicago, IL.
FAU - Carns, Mary
AU  - Carns M
AD  - Division of Rheumatology, Northwestern University, Feinberg School of Medicine,
      Chicago, IL.
FAU - Ramos, Paula S
AU  - Ramos PS
AD  - Division of Rheumatology, Medical University of South Carolina, Charleston, SC.
FAU - Silver, Richard M
AU  - Silver RM
AD  - Division of Rheumatology, Medical University of South Carolina, Charleston, SC.
FAU - Schiopu, Elena
AU  - Schiopu E
AD  - Division of Rheumatology, University of Michigan, Ann Arbor, MI.
FAU - Khanna, Dinesh
AU  - Khanna D
AD  - Division of Rheumatology, University of Michigan, Ann Arbor, MI.
FAU - Hsu, Vivien
AU  - Hsu V
AD  - Division of Rheumatology, Robert Wood Johnson University, New Brunswick, NJ.
FAU - Gordon, Jessica K
AU  - Gordon JK
AD  - Division of Rheumatology, Hospital for Special Surgery, New York, NY.
FAU - Gladue, Heather
AU  - Gladue H
AD  - Department of Rheumatology, Arthritis and Osteoporosis Consultants of the
      Carolinas, Charlotte, NC.
FAU - Saketkoo, Lesley A
AU  - Saketkoo LA
AD  - Division of Rheumatology, Tulane University School of Medicine, New Orleans, LA.
FAU - Criswell, Lindsey A
AU  - Criswell LA
AD  - Division of Rheumatology, University of California San Francisco, CA.
FAU - Derk, Chris T
AU  - Derk CT
AD  - Division of Rheumatology, University of Pennsylvania, Philadelphia, PA.
FAU - Trojanowski, Marcin A
AU  - Trojanowski MA
AD  - Division of Rheumatology, Boston University School of Medicine, Boston, MA.
FAU - Shanmugam, Victoria K
AU  - Shanmugam VK
AD  - Division of Rheumatology, George Washington University, Washington, DC.
FAU - Chung, Lorinda
AU  - Chung L
AD  - Division of Rheumatology, Stanford University School of Medicine, Stanford, CA.
FAU - Valenzuela, Antonia
AU  - Valenzuela A
AD  - Division of Rheumatology, Stanford University School of Medicine, Stanford, CA.
FAU - Jan, Reem
AU  - Jan R
AD  - Division of Rheumatology, University of Chicago Pritzker School of Medicine,
      Chicago, IL.
FAU - Goldberg, Avram
AU  - Goldberg A
AD  - Division of Rheumatology, New York University Langone Medical Center, New York,
      NY.
FAU - Remmers, Elaine F
AU  - Remmers EF
AD  - National Human Genome Research Institute.
FAU - Kastner, Daniel L
AU  - Kastner DL
AD  - National Human Genome Research Institute.
FAU - Wigley, Fredrick M
AU  - Wigley FM
AD  - Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore,
      MD.
FAU - Gourh, Pravitt
AU  - Gourh P
AD  - National Institute of Arthritis and Musculoskeletal and Skin Diseases, National
      Institutes of Health, Bethesda, MD, USA.
FAU - Boin, Francesco
AU  - Boin F
AD  - Division of Rheumatology, University of California San Francisco, CA.
LA  - eng
GR  - K23 AR061439/AR/NIAMS NIH HHS/United States
GR  - P50 AR054144/AR/NIAMS NIH HHS/United States
GR  - T32 AR048522/AR/NIAMS NIH HHS/United States
GR  - R03 AR065801/AR/NIAMS NIH HHS/United States
GR  - K01 AR067280/AR/NIAMS NIH HHS/United States
GR  - UL1 TR000062/TR/NCATS NIH HHS/United States
GR  - K24 AR063120/AR/NIAMS NIH HHS/United States
GR  - UL1 RR029882/RR/NCRR NIH HHS/United States
GR  - N01AR02251/AR/NIAMS NIH HHS/United States
GR  - R01 AR055258/AR/NIAMS NIH HHS/United States
GR  - P60 AR062755/AR/NIAMS NIH HHS/United States
PT  - Journal Article
PT  - Multicenter Study
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
SB  - AIM
SB  - IM
MH  - Adult
MH  - African Americans
MH  - Chromosome Mapping
MH  - Cohort Studies
MH  - Cross-Sectional Studies
MH  - Databases, Factual
MH  - Female
MH  - Humans
MH  - Male
MH  - Prevalence
MH  - Prospective Studies
MH  - Retrospective Studies
MH  - Scleroderma, Systemic/blood/*epidemiology/ethnology/physiopathology
MH  - Severity of Illness Index
MH  - Socioeconomic Factors
MH  - United States/epidemiology
PMC - PMC5758130
EDAT- 2018/02/03 06:00
MHDA- 2018/02/14 06:00
CRDT- 2018/02/03 06:00
PHST- 2018/02/03 06:00 [entrez]
PHST- 2018/02/03 06:00 [pubmed]
PHST- 2018/02/14 06:00 [medline]
AID - 10.1097/MD.0000000000008980 [doi]
AID - 00005792-201712220-00015 [pii]
PST - ppublish
SO  - Medicine (Baltimore). 2017 Dec;96(51):e8980. doi: 10.1097/MD.0000000000008980.