Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2023 Nov 1;35(6):324-333.
doi: 10.1097/BOR.0000000000000965. Epub 2023 Aug 13.

Raynaud phenomenon and microvasculopathy in systemic sclerosis: multi-modality imaging for diagnosis and evaluation

George Markousis-Mavrogenis  1 , Vasiliki-Kalliopi Bournia  2 , Petros P Sfikakis  2 , Sophie I Mavrogeni  1   3
Affiliations
Review

Raynaud phenomenon and microvasculopathy in systemic sclerosis: multi-modality imaging for diagnosis and evaluation

George Markousis-Mavrogenis et al. Curr Opin Rheumatol. .

Abstract

Purpose of review: To describe the clinical significance of and the diagnostic approach to Raynaud phenomenon (RP) in the peripheral extremities and the heart.

Recent findings: Nailfold capillaroscopy has recently been standardized in an expert consensus paper. Abnormal capillaroscopy in combination with specific autoantibody profiles and clinical signs are highly predictive of progression of RP to systemic sclerosis (SSc). Magnetic resonance imaging (MRI) can also perform tissue characterization of both the extremities and the heart. Microvascular wall abnormalities detected using nailfold capillaroscopy in patients with SSc may lead to deposition of erythrocyte-derived iron, due to microhemorrhages, which may predispose to fibrosis. MRI can assess the presence of iron using T2∗ measurements.

Summary: RP is a hallmark of the microvasculopathy in SSc and can affect both the peripheral extremities and the heart. Nailfold capillaroscopy is the current gold standard for the evaluation of the peripheral microvasculature. Other imaging modalities include thermography, laser Doppler-derived methods, 99m Tc-pertechnetate hand perfusion scintigraphy, power Doppler ultrasonography, dynamic optical coherence tomography, MRI, and photoacoustic imaging, but these are currently not widely used. Cardiac RP can be investigated with positron emission tomography or cardiovascular magnetic resonance, with the latter offering the additional possibility of tissue characterization and iron content quantification secondary to microhemorrhages.

PubMed Disclaimer

References

    1. Bournia VK, Fragoulis GE, Mitrou P, et al. All-cause mortality in systemic rheumatic diseases under treatment compared with the general population. RMD Open 2021; 7:e001694.
    1. Cutolo M, Soldano S, Smith V. Pathophysiology of systemic sclerosis: current understanding and new insights. Expert Rev Clin Immunol 2019; 15:753–764.
    1. Masi AT, Rodnan GP, Medsger TA Jr, et al. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Arthritis Rheum 1980; 23:581–590.
    1. Alexander EL, Firestein GS, Weiss JL, et al. Reversible cold-induced abnormalities in myocardial perfusion and function in systemic sclerosis. Ann Intern Med 1986; 105:661–668.
    1. Garner R, Kumari R, Lanyon P, et al. Prevalence, risk factors and associations of primary Raynaud's phenomenon: systematic review and meta-analysis of observational studies. BMJ Open 2015; 5:e006389.