Contemporary Management of Raynaud's Phenomenon and Digital Ischaemic Complications

Ariane L. Herrick

Disclosures

Curr Opin Rheumatol. 2011;23(6):555-561. 

In This Article

Principles of Management

The principles of current management of Raynaud's phenomenon, with and without ischaemic complications, are summarized in Fig. 2. The EULAR (European League Against Rheumatism) recommendations for the treatment of SSc[4] include Raynaud's phenomenon and digital ulcers. Drug treatment is indicated in those patients who do not respond to general measures. Calcium channel blockers[5,6] are the drugs of first choice, although adverse effects are common.[7] Sustained release preparations (e.g. sustained release nifedipine or amlodipine) are often better tolerated than short-acting preparations. If a calcium channel blocker is ineffective or only partially effective, then other vasoactive drugs may be added in or substituted (Fig. 2), although the evidence base in many instances is weak.[7] Angiotensin II receptor antagonists are now widely used: losartan reduced frequency and severity of Raynaud's attacks in an open study[8] including patients with both primary and secondary Raynaud's phenomenon. Angiotensin-converting enzyme (ACE) inhibitors are sometimes used, but a double-blind controlled trial[9] of 2–3 years of treatment with quinapril showed no benefit in 210 patients (186 with limited cutaneous SSc and 24 with antibody positive Raynaud's phenomenon), in terms of either Raynaud's symptoms or digital ulceration. There is some evidence in favour of alpha-blockade.[10] Selective serotonin reuptake inhibitors (SSRIs) were found to be beneficial in an open study[11] and may be better tolerated than the drugs already mentioned in patients prone to vasodilatory side-effects. Phosphodiesterase inhibitors (discussed in detail below) are being increasingly used, especially in patients with severe Raynaud's phenomenon who have not responded to other therapies. Topical nitrate therapy is currently not widely used but is being 'revisited' as discussed below. Intravenous iloprost[7,12,13] may be used in patients with severe Raynaud's phenomenon, but tends to be reserved for those with digital ulceration or critical digital ischaemia. Different dosage regimes for iloprost continue to be studied.[14]

Figure 2.

Flow-chart outlining the stepwise approach to contemporary management

For patients with SSc-spectrum disorders who progress to digital ulceration and/or critical ischaemia (these often co-exist), intensification of treatment is required:

  1. Digital ulceration (Fig. 1a). Digital ulcers are often extremely painful and may be infected. Therefore, analgesics and antibiotics are important aspects of management. Opiates may be required in the short term. Intravenous prostanoids are the mainstay of current management. The ET-1 receptor antagonist bosentan, discussed below, is now licensed for prevention of digital ulceration in patients with SSc and recurrent ulcers. Expert nursing care, with tissue viability input to optimize dressings, is an important aspect of management. Surgical debridement should be considered, especially if there is necrotic tissue present. Osteomyelitis can complicate digital ulceration and may be diagnosed at an early stage by magnetic resonance (MR) scanning, allowing prompt treatment with prolonged antibiotic therapy: this may save the digit.

  2. Critical ischaemia (Fig. 1b). A key point is to ensure that there is no concomitant disorder (large vessel disease, vasculitis, coagulopathy) requiring specific treatment. Intravenous prostanoids are currently first-line management. Given the increased platelet aggregation and pro-coagulant tendency of patients with SSc, consideration should be given to antiplatelet therapy, and in the short-term to anticoagulation, although the evidence base for either of these approaches is weak.

Digital (palmar) sympathectomy should be considered for patients unresponsive to the above measures, although this is a specialist procedure performed only in certain centres. There are now a number of reports describing favourable long-term outcomes, although numbers remain small.[15–17] Some patients with digital ulceration or critical ischaemia unresponsive to the above measures will require amputation.

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