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  • Review Article
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Detection of microvascular changes in systemic sclerosis and other rheumatic diseases

Nature Reviews Rheumatology volume 17, pages 665–677 (2021)Cite this article

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Abstract

Morphological and functional analysis of the microcirculation are objective outcome measures that are recommended for use in the presence of clinical signs of altered peripheral blood flow (such as Raynaud phenomenon), which can occur in systemic sclerosis (SSc) and other autoimmune rheumatic diseases. Several advanced non-invasive tools are available for monitoring the microcirculation, including nailfold videocapillaroscopy, which is the best-studied and most commonly used method for distinguishing and quantifying microvascular morphological alterations in SSc. Nailfold videocapillaroscopy can also be used alongside laser Doppler techniques to assist in the early diagnosis and follow-up of patients with dermatomyositis or mixed connective tissue disease. Power Doppler ultrasonography, which has been used for many years to evaluate the vascularity of synovial tissue in rheumatoid arthritis, is another promising tool for the analysis of skin and nailbed capillary perfusion in other autoimmune rheumatic diseases. Other emerging methods include raster-scanning optoacoustic mesoscopy, which offers non-invasive high-resolution 3D visualization of capillaries and has been tested in psoriatic arthritis and SSc. The principle functions and operative characteristics of several non-invasive tools for analysing microvascular changes are outlined in this Review, and the clinical roles of validated or tested imaging methods are discussed for autoimmune rheumatic diseases.

Key points

  • Careful detection of the microvasculature can be performed using a variety of advanced imaging methods, which have also enabled new insights into the pathophysiology of several autoimmune rheumatic diseases.

  • Nailfold videocapillaroscopy is the gold standard method for distinguishing between primary and secondary Raynaud phenomenon, through the identification of a ‘scleroderma’ pattern, and for quantifying differences in microvascular morphology.

  • The presence of a ‘scleroderma-like’ nailfold videocapillaroscopy pattern supports the diagnosis of autoimmune rheumatic diseases such as dermatomyositis, mixed connective tissue disease and antisynthetase syndrome.

  • Several non-invasive methods, including laser Doppler techniques, enable the detection and quantification of characteristic alterations in peripheral blood perfusion in a number of autoimmune rheumatic diseases.

  • Imaging of the microcirculation is recommended at least twice a year for patients with persistent Raynaud phenomenon, and also for the follow-up of patients with selected autoimmune rheumatic diseases.

  • Almost all the most common techniques for morphological and functional microvascular evaluation can be used in combinations and managed by rheumatologists, bringing diagnostic power to the rheumatologist’s imaging armamentarium.

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Fig. 1: Direct optical imaging of microvascular morphology.
Fig. 2: Optoacoustic imaging of microvascular morphology.
Fig. 3: Imaging of microvascular function.
Fig. 4: Alternative imaging tools for assessing peripheral blood flow.
Fig. 5: Nailfold capillaroscopic patterns in rheumatic diseases other than systemic sclerosis.
Fig. 6: Combined microvascular morphology and functional blood flow analysis.

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Acknowledgements

The authors thank C. Pizzorni, A. Sulli and S. Paolino for their continuous support and S. De Gregorio and E. Gotelli for their involvement in figure definition. M.C. and V.S. are members of and/or chair the EULAR Study Group on Microcirculation in Rheumatic Diseases, which makes the dissemination of research projects on this topic possible.

Author information

Authors and Affiliations

  1. Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DiMI), University of Genova–IRCCS San Martino Polyclinic Hospital, Genoa, Italy

    Maurizio Cutolo

  2. Department of Rheumatology, Ghent University Hospital, Ghent, Belgium

    Vanessa Smith

  3. Department of Internal Medicine, Ghent University, Ghent, Belgium

    Vanessa Smith

  4. Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Centre (IRC), Ghent, Belgium

    Vanessa Smith

Authors
  1. Maurizio Cutolo
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  2. Vanessa Smith
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Contributions

M.C. researched data for the article and wrote the article. V.S. reviewed and/or edited the manuscript before submission.

Corresponding author

Correspondence to Maurizio Cutolo.

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The authors declare no competing interests.

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Disclaimer

V.S. is a senior clinical investigator of the Research Foundation Flanders, Belgium (FWO; 1.8.029.20 N). The FWO was not involved in the study design, collection, analysis and interpretation of data, writing of the report or in the decision to submit the manuscript for publication.

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Nature Reviews Rheumatology thanks S. Lambova, J.-L. Cracowski and the other, anonymous, reviewer(s) for their contribution to the peer review of this work.

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Glossary

Telangiectasias

Also named spider veins, these are small, dilated blood vessels that can occur near the surface of the skin or mucous membranes.

Proximal–distal gradient

In healthy individuals, perfusion is typically high in the distal fingertips and low in the dorsum of the hands.

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Cutolo, M., Smith, V. Detection of microvascular changes in systemic sclerosis and other rheumatic diseases. Nat Rev Rheumatol 17, 665–677 (2021). https://doi.org/10.1038/s41584-021-00685-0

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