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Review
. 2017 Dec;9(12):317-329.
doi: 10.1177/1759720X17740074. Epub 2017 Nov 20.

Evidence-based management of Raynaud's phenomenon

Ariane L Herrick  1
Affiliations
Review

Evidence-based management of Raynaud's phenomenon

Ariane L Herrick. Ther Adv Musculoskelet Dis. 2017 Dec.

Abstract

Raynaud's phenomenon (RP) is relevant to the rheumatologist because it may signify an underlying connective tissue disease and also because it can be very challenging to treat, especially when it has progressed to digital ulceration or critical ischaemia. This review article discusses diagnosis (does this patient have an underlying connective tissue disease?), including the role for nailfold capillaroscopy, and treatment. Management of 'uncomplicated' RP is first described and then treatment of RP complicated by progression to digital ulceration or critical ischaemia, highlighting recent advances (including phosphodiesterase type 5 inhibition, and endothelin 1 receptor antagonism) and the evidence base underpinning these. Possible future therapies are briefly discussed.

Keywords: Raynaud’s phenomenon; critical ischaemia; digital ulceration; systemic sclerosis; treatment.

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Conflict of interest statement

Conflict of interest statement: ALH has done consultancy work for Actelion, served on a Data Safety Monitoring Board for Apricus, received research funding and speaker’s fees from Actelion, and speaker’s fees from GSK.

Figures

Figure 1.
Figure 1.
Digital pitting in a patient with systemic sclerosis.
Figure 2.
Figure 2.
(a) Normal and (b) abnormal nailfold capillaries, with dilated capillaries, distortion of the normal nailfold architecture and areas of avascularity.
Figure 3.
Figure 3.
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. Note that clinicians outside the UK might modify their approach depending on their access to therapies. ACE, angiotensin-converting enzyme; ARB, angiotensin receptor blocker: CCB, calcium channel blocker; PDE5, phosphodiesterase type 5; SSRI, selective serotonin reuptake inhibitor. Modified from Herrick.
Figure 4.
Figure 4.
Critical digital ischaemia of the thumb in a patient with systemic sclerosis and very severe digital vasculopathy (digital ulcers are also present).
Figure 5.
Figure 5.
Modification of the UK Scleroderma Study Group Best Practice Recommendations on the management of systemic sclerosis (SSc)-related digital ulceration. ERA, endothelin-1 receptor antagonist; PDE5, phosphodiesterase type 5. Modified from Herrick.
See this image and copyright information in PMC

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