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The Management of Pulmonary Arterial Hypertension in the Setting of Systemic Sclerosis

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Scleroderma

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Abstract

Pulmonary hypertension (PH) commonly complicates systemic sclerosis (SSc). PH related to SSc can develop in multiple ways and thus be classified in any of the five groups of pulmonary hypertension as defined by the World Health Organization. PH significantly impacts morbidity and mortality in SSc and, along with interstitial lung disease, is currently the leading cause of death in this population. While advances in therapeutics have led to improvements in symptoms, functional capacity, and outcomes in patients with pulmonary arterial hypertension (PAH), the response to therapy in PAH related to SSc (SSc-PAH) is, in general, less robust than other forms of PAH. Further, there are few randomized clinical trials of these therapies in other forms of PH, and thus, treatment in these populations is challenging. Newer therapeutic strategies, such as simultaneously targeting multiple pathways involved in the putative pathogenesis of the disease or utilizing inhaled therapies, may offer improved outcomes for SSc-PAH and lead to randomized trials in other forms of SSc-related PH. Focusing on supportive therapy, such as pulmonary rehabilitation, may also be important in the management of this complex disease.

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Authors and Affiliations

  1. Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, 21205, USA

    Stephen C. Mathai MD, MHS

  2. Department of Experimental, Diagnostic and Specialty Medicine-DIMES, University of Bologna, Via Massarenti 9, Bologna, 40138, Italy

    N. Nazzareno Galié MD

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  1. Stephen C. Mathai MD, MHS
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Correspondence to Stephen C. Mathai MD, MHS .

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Editors and Affiliations

  1. Feinberg School of Medicine, Northwestern University, Chicago, Illinois, USA

    John Varga

  2. Division of Medicine, Department of Inflammation, Centre for Rheumatology and Connective Tissue Diseases, UCL Division of Medicine, Royal Free Hospital, London, United Kingdom

    Christopher P. Denton

  3. Division of Rheumatology, Johns Hopkins University, Baltimore, Maryland, USA

    Fredrick M. Wigley

  4. Service de Rhumatologie A, INSERMU1016, Universit. Paris Descartes, Hôpital Cochin, Paris, France

    Yannick Allanore

  5. Department of Allergy and Rheumatology, Nippon Medical School, Graduate School of Medicine, Tokyo, Japan

    Masataka Kuwana

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Mathai, S.C., Galié, N.N. (2017). The Management of Pulmonary Arterial Hypertension in the Setting of Systemic Sclerosis. In: Varga, J., Denton, C., Wigley, F., Allanore, Y., Kuwana, M. (eds) Scleroderma. Springer, Cham. https://doi.org/10.1007/978-3-319-31407-5_28

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