• Medicine · Jul 2014

    Comparative Study

    Severity of systemic sclerosis-associated pulmonary arterial hypertension in African Americans.

    • Isabel Blanco, Stephen Mathai, Majid Shafiq, Danielle Boyce, M KolbToddT, Hala Chami, K HummersLauraL, Traci Housten, Neal Chaisson, L ZaimanAriA, M WigleyFredrickF, J TedfordRyanR, A KassDavidD, Rachel Damico, E GirgisRedaR, and M HassounPaulP.
    • 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.
    • Medicine (Baltimore). 2014 Jul 1; 93 (5): 177185177-185.

    AbstractAfrican 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.

      Pubmed     Free full text   Copy Citation     Plaintext  

      Add institutional full text...

    Notes

     
    Knowledge, pearl, summary or comment to share?
    300 characters remaining
    help        
    You can also include formatting, links, images and footnotes in your notes
    • Simple formatting can be added to notes, such as *italics*, _underline_ or **bold**.
    • Superscript can be denoted by <sup>text</sup> and subscript <sub>text</sub>.
    • Numbered or bulleted lists can be created using either numbered lines 1. 2. 3., hyphens - or asterisks *.
    • Links can be included with: [my link to pubmed](http://pubmed.com)
    • Images can be included with: ![alt text](https://bestmedicaljournal.com/study_graph.jpg "Image Title Text")
    • For footnotes use [^1](This is a footnote.) inline.
    • Or use an inline reference [^1] to refer to a longer footnote elseweher in the document [^1]: This is a long footnote..

    hide…