Current Approaches to the Treatment of Systemic-Sclerosis-Associated Pulmonary Arterial Hypertension (SSc-PAH)
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  • 作者：Vincent Sobanski ; David Launay ; Eric Hachulla ; Marc Humbert
  • 关键词：Pulmonary arterial hypertension ; Systemic sclerosis ; Connective tissue disease ; Treatment
  • 刊名：Current Rheumatology Reports
  • 出版年：2016
  • 出版时间：January 2016
  • 年：2016
  • 卷：18
  • 期：2
  • 全文大小：1,747 KB
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  • 作者单位：Vincent Sobanski (1) (2) (3) (4)
    David Launay (1) (2) (3) (4)
    Eric Hachulla (1) (2) (3) (4)
    Marc Humbert (5) (6) (7)

    1. U995 Lille Inflammation Research International Center, University Lille, Lille, France
    2. Département de Médecine Interne et Immunologie Clinique, CHU Lille, Lille, France
    3. Centre National de Référence Maladies Systémiques et Auto-Immunes Rares (Sclérodermie Systémique), Lille, France
    4. FHU Immune-Mediated Inflammatory Diseases and Targeted Therapies (IMMINeNT), Lille, France
    5. Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin Bicêtre, France
    6. Service de Pneumologie, Hôpital Bicêtre, AP-HP, Le Kremlin Bicêtre, France
    7. INSERM UMR S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
  • 刊物主题：Rheumatology;
  • 出版者：Springer US
  • ISSN：1534-6307
    • 文摘
    Pulmonary arterial hypertension (PAH) is a severe condition causing significant morbidity and mortality in patients with systemic sclerosis (SSc). Despite the use of specific treatments, SSc-PAH survival remains poorer than in idiopathic PAH (IPAH). Recent therapeutic advances in PAH show a lower magnitude of response in SSc-PAH and a higher risk of adverse events, as compared to IPAH. The multifaceted underlying mechanisms and the multisystem nature of SSc probably explain part of the worse outcomes in SSc-PAH compared to IPAH. This review describes the current management of SSc-PAH with an emphasis on the impact of the different organ involvements in the prognosis and treatment response. An earlier detection of PAH and a better characterization of the clinical phenotypes of SSc-PAH are warranted in clinical practice and future trials. Determinants of prognosis, surrogate markers of clinical improvement or worsening, and relevance of the common endpoints used in clinical trials should be evaluated in this specific population. A multidisciplinary approach in expert referral centers is mandatory for SSc-PAH management. Keywords Pulmonary arterial hypertension Systemic sclerosis Connective tissue disease Treatment

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