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Mechanisms of Vascular Disease

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Scleroderma

Abstract

Scleroderma (systemic sclerosis (SSc)) is to a large degree identified by the vascular features which may precede the onset of fibrosis by months or even years. Abnormalities in microvessel morphology and vascular dysfunction occur early and evolve into a distinctive vasculopathy that relentlessly advances in nearly all affected organs. This evidence suggests that endothelial cells (ECs) are the primary target in SSc and that the interactions of ECs with other cells and pathways, including cells of both the innate and adaptive immune systems, platelets and coagulation factors, vascular smooth muscle cells and fibroblasts, may take center stage in the disease pathogenesis. Therapeutically, the use of angiotensin-converting enzyme inhibitors for scleroderma renal crisis, endothelin receptor antagonists for pulmonary arterial hypertension (PAH), and phosphodiesterase inhibitors for PAH, Raynaud’s phenomenon, and digital ulcers has significantly improved the care of SSc patients. However, despite a substantial symptomatic improvement, regression of the vascular lesions has been difficult to achieve.

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Authors and Affiliations

  1. Section of Anatomy and Histology, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy

    Mirko Manetti PhD

  2. Division of Rheumatology and Immunology, University of Toledo Medical Center, Toledo, OH, USA

    Bashar Kahaleh MD

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  1. Mirko Manetti PhD
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Correspondence to Mirko Manetti PhD .

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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, HĂ´pital 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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Manetti, M., Kahaleh, B. (2017). Mechanisms of Vascular Disease. 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_16

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