Management of Raynaud’s Phenomenon and Digital Ulcers

Abstract

This review describes the principles of approach to, and management of, the patient with Raynaud’s phenomenon (RP). The first step is to establish whether RP is primary or secondary to an underlying cause. If RP is secondary, then this has implications for management: the cause may be treatable, and the RP is likely to be more severe than primary RP. If RP is secondary to systemic sclerosis (the type of RP which has been most researched), then the patient will have structural as well as functional vascular changes which may progress to digital ulceration and critical ischemia. Triggering factors should be avoided, and therefore, patient education is an important part of management. The approach to drug treatment is described for patients with mild, moderate and severe RP (severe meaning complicated by the development of digital ulcers or critical ischemia). Surgical interventions are briefly described, bearing in mind that only a minority of patients require surgery. The final section of the review discusses novel (possible future) therapies. Raynaud’s phenomenon (RP) is the clinical manifestation of disrupted regulation of the peripheral vasculature that is critical for tissue nutrition and body thermoregulation. While not all patients develop ulceration or critical ischemia, managing RP is most important to improve quality of life (QOL) and prevent secondary complications. Non-drug therapy should be used in every case including cold avoidance, elimination of smoking, stopping aggravating drugs and providing support and education to reduce stress. Drug therapy is helpful to improve QOL and prevent secondary complications, but ideal studies are lacking to provide solid evidence for best options. We prefer calcium channel blockers alone for moderate cases and prostaglandins for severe RP. Combination therapy such as a calcium channel blocker and a phosphodiesterase type 5 (PDE5) inhibitor appears helpful in complex cases. While botulinum toxin (Botox) injections are becoming popular, studies are needed to provide evidence for this approach. Patients with systemic sclerosis (SSc; scleroderma) and secondary digital ulcers or those with critical ischemia require more intense therapy with both vasodilation therapy and agents that have the potential to prevent further vascular insult. This is an area in need of further study, but the use of intermittent prostacyclin, antiplatelet therapy, or use of an endothelin receptor antagonist are current options. Digital sympathectomy may provide benefit (but which may only be transient) for critical ischemia. Macrovascular disease can coexist with SSc-related microangiopathy, and comorbid conditions need to be addressed with surgical correction of occlusive disease if feasible. There is a need for new agents that directly target the disease process.