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

  • Vascular Disease
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Opinion statement

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.

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Acknowledgment

The author is grateful to Ms. Marcia Wyman, PharmD, drug information pharmacist at the Cleveland Clinic, for providing the prices of the individual drugs.

Disclosure

Dr. Chatterjee is a member of the Scientific Advisory Board for and has also received grant support from Gilead Sciences, Inc. He is an investigator for clinical trials run by United Therapeutics Corporation, MedImmune, and MediQuest Therapeutics.

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  1. Department of Rheumatic and Immunologic Diseases, Orthopedic and Rheumatology Institute, Cleveland Clinic, 9500 Euclid Avenue, Desk A50, Cleveland, OH, 44195, USA

    Soumya Chatterjee MD, MS, FRCP

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Correspondence to Soumya Chatterjee MD, MS, FRCP.

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Chatterjee, S. Management of Raynaud’s Phenomenon in the Patient with Connective Tissue Disease. Curr Treat Options Cardio Med 12, 185–204 (2010). https://doi.org/10.1007/s11936-010-0065-x

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