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Review
. 2019 Jun;4(2):89-101.
doi: 10.1177/2397198319826467. Epub 2019 Feb 13.

Raynaud's phenomenon

Ariane L Herrick  1   2
Affiliations
Review

Raynaud's phenomenon

Ariane L Herrick. J Scleroderma Relat Disord. 2019 Jun.

Abstract

Raynaud's phenomenon can be either primary (idiopathic) or secondary to underlying disease including systemic sclerosis. Primary Raynaud's phenomenon is very common, affecting approximately 3%-5% of the general population. Although much rarer, systemic sclerosis-related Raynaud's phenomenon can be particularly severe, progressing to digital ulceration in approximately 50% of patients. Raynaud's phenomenon can have a major impact on quality of life. This review has a focus on the systemic sclerosis-related Raynaud's phenomenon (which is the most researched form of Raynaud's phenomenon and probably the most challenging to treat) and on recent advances. Epidemiology (including transition from 'isolated' to systemic sclerosis-related Raynaud's phenomenon), pathogenesis, diagnosis and assessment are discussed, followed by the treatment of both 'uncomplicated' and 'complicated' Raynaud's phenomena (i.e. Raynaud's phenomenon which has progressed to digital ulceration and/or critical ischaemia). Finally, some of the major challenges for the next 5-10 years are highlighted.

Keywords: Raynaud’s phenomenon; Systemic sclerosis; assessment; diagnosis; management.

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Conflict of interest statement

Declaration of conflicting interests: A.L.H. received research funding from Actelion.

Figures

Figure 1.
Figure 1.
Nailfold capillaroscopy (left panel) is normal. Thermography showed that although the fingertips were cool at a room temperature of 23°C (middle panel), at 30°C (right panel), there were no significant persistent temperature gradients (as defined by a fingertip more than 1°C cooler than the dorsum of hand) along any of the fingers. This pattern is consistent with primary RP.,
Figure 2.
Figure 2.
Capillaroscopy (left index finger) shows an enlarged capillary and haemorrhages. Thermography shows cool fingertips at a room temperature of 23°C (middle panel), but not persisting at 30°C.
Figure 3.
Figure 3.
Nailfold capillaroscopy was very abnormal with dilated loops, avascularity, distortion of the normal nailfold architecture and haemorrhages. Thermographic testing showed that at a room temperature of 30°C, there were persisting temperature gradients (fingertips >1°C cooler than dorsum of hand) along several of the fingers. This pattern is consistent with SSc.,
Figure 4.
Figure 4.
Mobile phone photographs of (a) hands and (b) foot (same patient) showing the ‘white’ phase of RP.
Figure 5.
Figure 5.
Puffy fingers – a ‘red flag’ for SSc in the patient presenting with RP.
Figure 6.
Figure 6.
Modification of the UK Scleroderma Study Group best practice recommendations on the management of Raynaud’s phenomenon. Phosphodiesterase inhibition has been ‘moved up’ the original pathway to be positioned along with other oral vasodilator therapies. ACE: angiotensin-converting enzyme; ARB: angiotensin receptor blocker: CCB: calcium channel blockers; PDE5: phosphodiesterase type 5; SSRI: selective serotonin reuptake inhibitor.
Figure 7.
Figure 7.
Modification of the UK Scleroderma Study Group best practice recommendations on the management of SSc-related digital ulceration. PDE5: phosphodiesterase type 5.
See this image and copyright information in PMC

References

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