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Systemic Vascular Disease

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Scleroderma

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Abstract

Vascular disease, predominantly affecting the microcirculation and small arteries, invariably develops in systemic sclerosis. The most obvious clinical consequences of this include Raynaud’s phenomenon, digital ischaemic ulcers, telangiectasia, renovascular complications and pulmonary hypertension. However, it almost certainly has a pivotal role in the pathogenesis of this disease. Endothelial cell injury and vascular changes appear to precede other features of the disease and the subsequent vasculopathy is both dynamic and progressive. The extent and severity of vascular disease are major factors in determining the overall prognosis in individual cases. (Steen VD, Medsger Jr TA. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum. 2000;43(11):2437–44.)1

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Authors and Affiliations

  1. Bangor University, School of Medical Sciences, Bangor, Gwynedd, UK

    Peter Maddison MD, PhD

  2. Medicine, Biomedicine and Rheumatology AVC, University of Florence, Florence, Italy

    Marco Matucci-Cerinic MD, FRCP

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  1. Peter Maddison MD, PhD
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  2. Marco Matucci-Cerinic MD, FRCP
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Correspondence to Peter Maddison MD, PhD .

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Editors and Affiliations

  1. Feinberg School of Medicine, Northwestern University, E. Huron Street 240, Chicago, 60611-2909, Illinois, USA

    John Varga

  2. Royal Free and University College, Medical School, University College London, Rowland Street, London, NW3 2PF, United Kingdom

    Christopher P. Denton

  3. School of Medicine, Div. Rheumatology, Johns Hopkins University, Eastern Avenue 5200, Baltimore, 21224, Maryland, USA

    Fredrick M. Wigley

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Maddison, P., Matucci-Cerinic, M. (2012). Systemic Vascular Disease. In: Varga, J., Denton, C., Wigley, F. (eds) Scleroderma. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-5774-0_28

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