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Epidemiology and Risk Factors for Pulmonary Hypertension in Systemic Sclerosis

  • SCLERODERMA (J VARGA, SECTION EDITOR)
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Abstract

Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by vasculopathy and excessive collagen production leading to fibrosis of the skin and internal organs. SSc patients are at risk of developing pulmonary hypertension (PH), a debilitating, progressive condition of the pulmonary vasculature that leads to right heart failure and death. This review is an updated summary of the epidemiology and risk factors for PH in SSc. We describe the current literature examining the incidence, prevalence, and demographic and clinical risk factors associated with PH in SSc. We also discuss classical and novel autoantibodies and potential biomarkers that may be helpful in the assessment of risk and prognosis of PH in SSc patients. The ultimate objective in understanding the risk of developing PH in SSc is early diagnosis and early initiation of appropriate therapy with the hope for improved outcomes for patients with SSc-PH.

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Disclosure

Dr Chung has served as a consultant for Gilead Sciences, has received speakers’ honoraria from Gilead Sciences and Actelion Pharmaceuticals, and has had travel/accommodation expenses covered/reimbursed by Gilead Sciences and Actelion Pharmaceuticals. Dr Yaqub reported no potential conflicts of interest relevant to this article.

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Authors and Affiliations

  1. Division of Immunology and Rheumatology, Stanford University, Stanford, CA, USA

    Aaliya Yaqub & Lorinda Chung

  2. Veteran Affairs Palo Alto Health Care System, Palo Alto, CA, USA

    Lorinda Chung

  3. Division of Immunology and Rheumatology, Stanford University School of Medicine, 1000 Welch Road, #203, Palo Alto, CA, 94304, USA

    Aaliya Yaqub

  4. Division of Immunology and Rheumatology, Stanford University School of Medicine, Palo Alto Veterans Affairs Health Care System, 3801 Miranda Avenue, Room B2-139, Palo Alto, CA, 94305, USA

    Lorinda Chung

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  1. Aaliya Yaqub
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  2. Lorinda Chung
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Correspondence to Lorinda Chung.

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This article is part of the Topical Collection on Scleroderma

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Yaqub, A., Chung, L. Epidemiology and Risk Factors for Pulmonary Hypertension in Systemic Sclerosis. Curr Rheumatol Rep 15, 302 (2013). https://doi.org/10.1007/s11926-012-0302-2

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  • DOI: https://doi.org/10.1007/s11926-012-0302-2

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