Severity of Systemic Sclerosis-Associated Pulmonary Arterial Hypertension in African Americans

Medicine (Baltimore). 2014 Jul;93(5):177-85. doi: 10.1097/MD.0000000000000032.

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.

Publication types

  • Comparative Study
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • African Americans
  • Autoantibodies / blood
  • Cardiac Catheterization / methods
  • Echocardiography / methods
  • European Continental Ancestry Group
  • Exercise Test / methods
  • Familial Primary Pulmonary Hypertension
  • Female
  • Humans
  • Hypertension, Pulmonary* / blood
  • Hypertension, Pulmonary* / diagnosis
  • Hypertension, Pulmonary* / ethnology
  • Hypertension, Pulmonary* / etiology
  • Hypertension, Pulmonary* / physiopathology
  • Kaplan-Meier Estimate
  • Male
  • Middle Aged
  • Natriuretic Peptide, Brain / blood
  • Outcome Assessment, Health Care
  • Peptide Fragments / blood
  • Prevalence
  • Prognosis
  • Scleroderma, Systemic* / blood
  • Scleroderma, Systemic* / complications
  • Scleroderma, Systemic* / diagnosis
  • Scleroderma, Systemic* / ethnology
  • Scleroderma, Systemic* / physiopathology
  • Severity of Illness Index
  • United States / epidemiology
  • Ventricular Dysfunction, Right / etiology
  • Ventricular Dysfunction, Right / physiopathology

Substances

  • Autoantibodies
  • Peptide Fragments
  • pro-brain natriuretic peptide (1-76)
  • Natriuretic Peptide, Brain