title: Severity of systemic sclerosis-associated pulmonary arterial hypertension in African Americans.
abstract: African Americans (AA) with systemic sclerosis (SSc) have a worse prognosis compared to Americans of European descent (EA). We conducted the current study to test the hypothesis that AA patients with SSc have more severe disease and poorer outcomes compared to EA patients when afflicted with pulmonary arterial hypertension (PAH). We studied 160 consecutive SSc patients with PAH diagnosed by right heart catheterization, comparing demographics, hemodynamics, and outcomes between AA and EA patients. The cohort included 29 AA and 131 EA patients with similar baseline characteristics except for increased prevalence of diffuse SSc in AA. AA patients had worse functional class (FC) (80% FC III-IV vs 53%; p = 0.02), higher brain natriuretic peptide (NT-pro-BNP) (5729 ± 9730 pg/mL vs 1892 ± 2417 pg/mL; p = 0.02), more depressed right ventricular function, a trend toward lower 6-minute walk distance (263 ± 111  m vs 333 ± 110  m; p = 0.07), and worse hemodynamics (cardiac index 1.95 ± 0.58 L/min/m vs 2.62 ± 0.80 L/min/m; pulmonary vascular resistance 10.3 ± 6.2 WU vs 7.6 ± 5.0 WU; p  < 0.05) compared with EA patients. Kaplan-Meier survival estimates for AA and EA patients, respectively, were 62% vs 73% at 2 years and 26% vs 44% at 5 years (p  > 0.05). In conclusion, AA patients with SSc-PAH are more likely to have diffuse SSc and to present with significantly more severe PAH compared with EA patients. AA patients also appear to have poorer survival, though larger studies are needed to investigate this association definitively.
authors.affiliation: Divisions of Pulmonary and Critical Care Medicine (IB, SCM, DB, TMK, HC, TH, NC, ALZ, RD, REG, PMH), General Internal Medicine (MS, FMW), Rheumatology (LKH), and Cardiology (RJT, DAK), Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) (IB), Barcelona, Spain; and Centro de Investigación Biomédica en Red de Enfermedades Respiratorias (CIBERES) (IB), Spain.
authors.forename: Isabel
authors.initials: I
authors.lastname: Blanco
authors.forename: Stephen
authors.initials: S
authors.lastname: Mathai
authors.forename: Majid
authors.initials: M
authors.lastname: Shafiq
authors.forename: Danielle
authors.initials: D
authors.lastname: Boyce
authors.forename: Todd
authors.initials: T
authors.lastname: M Kolb
authors.forename: Hala
authors.initials: H
authors.lastname: Chami
authors.forename: Laura
authors.initials: L
authors.lastname: K Hummers
authors.forename: Traci
authors.initials: T
authors.lastname: Housten
authors.forename: Neal
authors.initials: N
authors.lastname: Chaisson
authors.forename: Ari
authors.initials: A
authors.lastname: L Zaiman
authors.forename: Fredrick
authors.initials: F
authors.lastname: M Wigley
authors.forename: Ryan
authors.initials: R
authors.lastname: J Tedford
authors.forename: David
authors.initials: D
authors.lastname: A Kass
authors.forename: Rachel
authors.initials: R
authors.lastname: Damico
authors.forename: Reda
authors.initials: R
authors.lastname: E Girgis
authors.forename: Paul
authors.initials: P
authors.lastname: M Hassoun
mesh_terms: D000328:Adult; D001741:Black or African American; D001323:Autoantibodies / Q000097:blood; D006328:Cardiac Catheterization / Q000379:methods; D004452:Echocardiography / Q000379:methods; D005080:Exercise Test / Q000379:methods; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000175:diagnosis / Q000208:ethnology / Q000209:etiology / Q000503:physiopathology; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000097:blood; D017063:Outcome Assessment, Health Care; D010446:Peptide Fragments / Q000097:blood; D015995:Prevalence; D011379:Prognosis; D012595:Scleroderma, Systemic / Q000097:blood / Q000150:complications / Q000175:diagnosis / Q000208:ethnology / Q000503:physiopathology; D012720:Severity of Illness Index; D014481:United States / Q000453:epidemiology; D018497:Ventricular Dysfunction, Right / Q000209:etiology / Q000503:physiopathology; D044465:White People
chemical_list: D001323:Autoantibodies; D010446:Peptide Fragments; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain