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Assessing microvascular changes in systemic sclerosis diagnosis and management

Nature Reviews Rheumatology volume 6, pages 578–587 (2010)Cite this article

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Abstract

Microvascular damage and dysfunction represent the earliest morphological and functional markers of systemic sclerosis (SSc), a progressive connective tissue disease characterized by vascular abnormalities and diffuse fibrosis in the skin and internal organs. These early microvascular changes are clinically mirrored by Raynaud phenomenon, which can be primary (idiopathic) or secondary to several different conditions including SSc. Morphological and functional assessment of the cutaneous microvasculature have crucial implications for diagnosis, prognosis and therapy in SSc and secondary Raynaud phenomenon. Most importantly, imaging with nailfold videocapillaroscopy (NVC) enables the early differentiation between primary and secondary Raynaud phenomenon by identifying morphological patterns specific to various stages of SSc ('early', 'active' and 'late' patterns); the inclusion of these NVC patterns could increase the sensitivity of classification criteria for SSc. Findings on NVC are also markers of SSc severity and progression, as reduced capillary density has been associated with a high risk of developing digital skin ulcers and pulmonary arterial hypertension. Laser Doppler imaging and thermal imaging demonstrate the dysfunctional cutaneous blood flow in response to cold stimuli. Therapies targeting underlying vascular disease in SSc have been successfully designed to improve the symptoms of Raynaud phenomenon and to reduce ischemic injury to involved organs, and NVC patterns have been found to improve following targeted therapy; however, treatment of later fibrosis remains a challenge.

Key Points

  • Systemic sclerosis (SSc) is a complex systemic connective tissue disease characterized by vasculopathy and progressive involvement of the skin and internal organs with diffuse fibrosis

  • Raynaud phenomenon as a clinical marker of altered microvascular structure can be primary (idiopathic) or secondary to various conditions, in particular SSc

  • Nailfold videocapillaroscopy (NVC) represents the best and safest method to analyze microvascular abnormalities in SSc, and enables the early differential diagnosis between primary and secondary Raynaud phenomenon

  • The dysfunctional cutaneous blood flow in patients with both SSc and secondary RP in response to temperature stimuli have been demonstrated by use of laser Doppler imaging techniques

  • Reduced capillary density on NVC correlates with a high risk of developing digital skin ulcers and the presence of pulmonary arterial hypertension, and can therefore be used as a marker of SSc severity and progression

  • Therapies targeting underlying vascular disease in SSc improve symptoms of Raynaud phenomenon and reduce ischemic injury to involved tissue and organs; however, targeted treatment of fibrosis remains a challenge

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Figure 1: Common clinical aspects of SSc.
Figure 2: The videocapillaroscope.
Figure 3: Markers of scleroderma patterns.
Figure 4: Nailfold videocapillaroscopic patterns of scleroderma (magnification × 200).
Figure 5: Laser Doppler imaging of cutaneous blood flow in secondary Raynaud phenomenon and normal conditions.
Figure 6: Thermal imaging of cutaneous blood vessel function in secondary Raynaud phenomenon.

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Acknowledgements

Charles P. Vega, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the MedscapeCME-accredited continuing medical education activity associated with this article.

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Authors and Affiliations

  1. Department of Internal Medicine, Research Laboratory and Academic Unit of Clinical Rheumatology, University of Genova, Viale Benedetto XV, Genova, 6-16132, Italy

    Maurizio Cutolo & Alberto Sulli

  2. Department of Rheumatology, Ghent University, De Pintelaan 185, Ghent, 9000, Belgium

    Vanessa Smith

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A. Sulli researched the data for the article. V. Smith and M. Cutolo provided substantial contributions to discussions of the content and reviewed and/or edited the manuscript before submission. M. Cutolo wrote the article.

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Correspondence to Maurizio Cutolo.

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Cutolo, M., Sulli, A. & Smith, V. Assessing microvascular changes in systemic sclerosis diagnosis and management. Nat Rev Rheumatol 6, 578–587 (2010). https://doi.org/10.1038/nrrheum.2010.104

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