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. Morgan; Ami A. Shah; Maureen D. Mayes; Robyn T. Domsic; Thomas A. Medsger; Virginia D. Steen; John Varga; Mary Carns; Paula S. Ramos; Richard M. Silver; Elena Schiopu; DInesh Khanna; Vivien Hsu; Jessica K. Gordon; Heather Gladue; Lesley A. Saketkoo; Lindsey A. Criswell; Chris T. Derk; Marcin A. Trojanowski; Victoria K. Shanmugam; Lorinda Chung; Antonia Valenzuela; Reem Jan; Avram Goldberg; Elaine F. Remmers; Daniel L. Kastner; Fredrick M. Wigley; Pravitt Gourh; Francesco Boin

doi:10.1097/MD.0000000000008980

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. Morgan, Ami A. Shah, Maureen D. Mayes, Robyn T. Domsic, Thomas A. Medsger, Virginia D. Steen, John Varga, Mary Carns, Paula S. Ramos, Richard M. Silver, Elena Schiopu, DInesh Khanna, Vivien Hsu, Jessica K. Gordon, Heather Gladue, Lesley A. Saketkoo, Lindsey A. Criswell, Chris T. Derk, Marcin A. Trojanowski, Victoria K. ShanmugamLorinda Chung, Antonia Valenzuela, Reem Jan, Avram Goldberg, Elaine F. Remmers, Daniel L. Kastner, Fredrick M. Wigley, Pravitt Gourh, Francesco Boin^*

^*Corresponding author for this work

Dermatology

Research output: Contribution to journal › Article › peer-review

77 Scopus citations

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.

Original language	English (US)
Article number	e8980
Journal	Medicine (United States)
Volume	96
Issue number	51
DOIs	https://doi.org/10.1097/MD.0000000000008980
State	Published - Dec 1 2017

Funding

Funding: The GRASP consortium was supported by research funding from the Scleroderma Research Foundation and the Intramural Research Programs of the National Human Genome Research Institute and the National Institute of Arthritis and Musculoskeletal and Skin Diseases. Dr Dinesh Khanna was supported by grants from NIAMS of the NIH K24 AR063120, has received investigator-initiated grants and acts as a consultant to Actelion, BMS, Bayer, Corbus, Cytori, ChemoMab, Eicos, GSK, Genentech/Roche, Sanofi-Aventis, and UCB. Dr Maureen Mayes was supported by grants from NIAMS of the NIH Centers of Research Translation P50-AR054144, NIH grant N01-AR-02251 and R01-AR-055258; and the Department of Defense Congressionally Directed Medical Research Program W81XWH-07–1–011 and WX81XWH-13–1-0452. Dr Chris Derk was supported by research funding from Gilead, Actelion and Cytori. Dr Paul Ramos was supported by grants from the NIH K01 AR067280, R03 AR065801, P60 AR062755, and the South Carolina Clinical and Translational Research Institute, with an academic home at the Medical University of South Carolina, through NIH Grants numbers UL1 RR029882 and UL1 TR000062. Dr Ami Shah was supported by NIAMS of the NIH under Award Number K23AR061439. Dr Nadia Morgan was supported by the National Institute of Arthritis, Musculoskeletal and Skin Diseases (NIAMS) of the National Institutes of Health (NIH) under Award Number T32AR048522 and the Rheumatology Research Foundation Scientist Development Award. Dr Richard Silver was supported by grants from the NIH P60 AR062755 and the South Carolina Clinical and Translational Research Institute, with an academic home at the Medical University of South Carolina, through NIH Grants numbers UL1 RR029882 and UL1 TR000062.

Keywords

African Americans
autoantibodies
systemic sclerosis

ASJC Scopus subject areas

General Medicine

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10.1097/MD.0000000000008980

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Morgan, N. D., Shah, A. A., Mayes, M. D., Domsic, R. T., Medsger, T. A., Steen, V. D., Varga, J., Carns, M., Ramos, P. S., Silver, R. M., Schiopu, E., Khanna, DI., Hsu, V., Gordon, J. K., Gladue, H., Saketkoo, L. A., Criswell, L. A., Derk, C. T., Trojanowski, M. A., ... Boin, F. (2017). 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. Medicine (United States), 96(51), Article e8980. https://doi.org/10.1097/MD.0000000000008980

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title = "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.",

keywords = "African Americans, autoantibodies, systemic sclerosis",

author = "Morgan, {Nadia D.} and Shah, {Ami A.} and Mayes, {Maureen D.} and Domsic, {Robyn T.} and Medsger, {Thomas A.} and Steen, {Virginia D.} and John Varga and Mary Carns and Ramos, {Paula S.} and Silver, {Richard M.} and Elena Schiopu and DInesh Khanna and Vivien Hsu and Gordon, {Jessica K.} and Heather Gladue and Saketkoo, {Lesley A.} and Criswell, {Lindsey A.} and Derk, {Chris T.} and Trojanowski, {Marcin A.} and Shanmugam, {Victoria K.} and Lorinda Chung and Antonia Valenzuela and Reem Jan and Avram Goldberg and Remmers, {Elaine F.} and Kastner, {Daniel L.} and Wigley, {Fredrick M.} and Pravitt Gourh and Francesco Boin",

note = "Publisher Copyright: {\textcopyright} 2017 the Author(s).",

year = "2017",

month = dec,

day = "1",

doi = "10.1097/MD.0000000000008980",

language = "English (US)",

volume = "96",

journal = "Medicine (United States)",

issn = "0025-7974",

publisher = "Lippincott Williams and Wilkins",

number = "51",

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Morgan, ND, Shah, AA, Mayes, MD, Domsic, RT, Medsger, TA, Steen, VD, Varga, J, Carns, M, Ramos, PS, Silver, RM, Schiopu, E, Khanna, DI, 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 2017, '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', Medicine (United States), vol. 96, no. 51, e8980. https://doi.org/10.1097/MD.0000000000008980

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, Nadia D.; Shah, Ami A.; Mayes, Maureen D. et al.
In: Medicine (United States), Vol. 96, No. 51, e8980, 01.12.2017.

Research output: Contribution to journal › Article › peer-review

TY - JOUR

T1 - Clinical and serological features of systemic sclerosis in a multicenter African American cohort

T2 - Analysis of the genome research in African American scleroderma patients clinical database

AU - Morgan, Nadia D.

AU - Shah, Ami A.

AU - Mayes, Maureen D.

AU - Domsic, Robyn T.

AU - Medsger, Thomas A.

AU - Steen, Virginia D.

AU - Varga, John

AU - Carns, Mary

AU - Ramos, Paula S.

AU - Silver, Richard M.

AU - Schiopu, Elena

AU - Khanna, DInesh

AU - Hsu, Vivien

AU - Gordon, Jessica K.

AU - Gladue, Heather

AU - Saketkoo, Lesley A.

AU - Criswell, Lindsey A.

AU - Derk, Chris T.

AU - Trojanowski, Marcin A.

AU - Shanmugam, Victoria K.

AU - Chung, Lorinda

AU - Valenzuela, Antonia

AU - Jan, Reem

AU - Goldberg, Avram

AU - Remmers, Elaine F.

AU - Kastner, Daniel L.

AU - Wigley, Fredrick M.

AU - Gourh, Pravitt

AU - Boin, Francesco

PY - 2017/12/1

Y1 - 2017/12/1

N2 - 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.

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.

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KW - autoantibodies

KW - systemic sclerosis

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AN - SCOPUS:85039731967

SN - 0025-7974

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JO - Medicine (United States)

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M1 - e8980

ER -

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

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