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Angiogenesis and Vasculopathy in Systemic Sclerosis: Evolving Concepts

  • SCLERODERMA (J VARGA, SECTION EDITOR)
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Abstract

Systemic sclerosis (scleroderma [SSc]) is a multifactorial disease characterized by inflammation, extensive and progressive fibrosis, and multiple vasculopathies. The vascular manifestations can be seen early in the pathogenesis of the disease and include malformed capillaries, Raynaud’s phenomenon, and digital ulcers. As the disease progresses, the vasculopathy proceeds to significant clinical manifestations, including renal crisis and pulmonary arterial hypertension. Moreover, later stages of the disease are marked by increasingly avascular areas. Despite the obliteration of microvascular structures, compensatory vasculogenesis and angiogenesis do not occur normally. This is in spite of a general increase in many potent angiogenic factors. Recent studies are beginning to examine this paradox and subsequent paucity of an angiogenic response in SSc. In this review, we discuss these findings and examine the role that chemokine and growth factor receptors, proteases, adhesion molecules, and transcription factors play in the dysregulation of angiogenesis in SSc.

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Acknowledgments

This work was supported by the National Institutes of Health (grant no. HL094017 to Dr. Rabquer), the Office of Research and Development, Medical Research Service, Department of Veterans Affairs, the Frederick G.L. Huetwell and William D. Robinson, MD, Professorship in Rheumatology, and by the Scleroderma Foundation (Mark Flapan Award).

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No potential conflicts of interest relevant to this article were reported.

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Authors and Affiliations

  1. Department of Internal Medicine, University of Michigan Medical School, 4388 BSRB, 109 Zina Pitcher Drive, Ann Arbor, MI, 48109-2200, USA

    Bradley J. Rabquer

  2. Department of Veterans Affairs, VA Medical Service, 2215 Fuller Road, Ann Arbor, MI, 48105, USA

    Alisa E. Koch

  3. Department of Internal Medicine, University of Michigan Medical School, 4045 BSRB, 109 Zina Pitcher Drive, Ann Arbor, MI, 48109-2200, USA

    Alisa E. Koch

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  1. Bradley J. Rabquer
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Correspondence to Alisa E. Koch.

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Rabquer, B.J., Koch, A.E. Angiogenesis and Vasculopathy in Systemic Sclerosis: Evolving Concepts. Curr Rheumatol Rep 14, 56–63 (2012). https://doi.org/10.1007/s11926-011-0219-1

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