Management of Raynaud’s Phenomenon in the Patient with Connective Tissue Disease

Abstract

Raynaud's phenomenon is characterized by intense vasospasm of digital arteries on cold exposure or with emotional stress, leading to well-defined color changes of digital skin. It may be primary (Raynaud's disease) or secondary to an underlying condition, including autoimmune rheumatic diseases. Although Raynaud's disease is predominantly a vasospastic condition, Raynaud's phenomenon in connective tissue diseases often is a result of an underlying vaso-occlusive process. As a result, the manifestations are more severe and persistent and often warrant pharmacologic therapy. Dihydropyridine calcium channel blockers are by far the most commonly studied and prescribed class of agents for the treatment of Raynaud's phenomenon. There is some evidence for the efficacy of other classes of drugs, such as topical nitrates, α-antagonists, angiotensin receptor blockers, selective serotonin reuptake inhibitors, and pentoxifylline. However, the data on the efficacy of these agents are not as convincing, and they are not proven to be more effective than calcium channel blockers. Hence, their place in the therapy of Raynaud's phenomenon is limited to patients who fail to respond adequately to or are unable to tolerate calcium channel blockers. More expensive second-line agents, such as phosphodiesterase-5 inhibitors, endothelin receptor antagonists, and intravenous prostanoids, are reserved for refractory cases of secondary Raynaud's phenomenon with severe digital ischemia leading to ulceration or gangrene. These drugs may be used in isolation or as adjunct therapy to the first-line agents. Chemical and/or surgical sympathectomy may be considered if sympathetically driven digital ischemia is severe and resistant to pharmacologic intervention. These procedures may temporarily reverse the digital ischemia and help tide over the crisis, whereas the improvement thus achieved can be maintained by continuing medical therapy. In cases of ischemic digital ulceration, it is important to achieve adequate analgesia and to identify and treat superadded infection.