Management of Raynaud Phenomenon and Digital Ulcers in Scleroderma

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Key points

  • The pathophysiology of Raynaud phenomenon (RP) and digital ulcers in scleroderma is complex, involving both vasospasm and structural disease of the vasculature.

  • Therapy for RP and digital ulcers should involve both nonpharmacologic and pharmacologic treatments.

  • Pharmacologic therapy for RP should include a combination of vasoactive agents that can reverse vasoconstriction and address biological pathways to prevent the progression of the underlying vasculopathy.

  • Digital ulcers are a common

Background

Raynaud phenomenon (RP) is one of the most common clinical manifestations of scleroderma, experienced by 90% to 98% of patients, usually as the first symptom in the course of the disease (Box 1, Table 1).1 The new 2013 classification criteria of the American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) now recognizes the importance of RP by including it as a feature to confirm a diagnosis of scleroderma.2 RP often predates other symptoms and signs by several years,

Digital ulcers

Digital ulcers are ischemic lesions representing end organ damage from the vascular disease of scleroderma. It is defined as an area with a visually discernible depth and a loss of continuity of epithelial coverage. The ischemic ulcer may be denuded or covered by necrotic tissue or scab. The prevalence of digital ulceration in scleroderma has been estimated at 10% to 54%32, 33, 34 and was about 15% in a recently published systematic review of the literature.35

Certain subgroups of patients are

Macrovascular disease in scleroderma

The understanding of scleroderma as a systemic vasculopathy has increased the recognition of macrovascular disease. Peripheral vascular disease (PVD) was the earliest form of macrovascular disease recognized in scleroderma.42 PVD can add to the compromise of peripheral blood flow and worsen microvascular manifestations. It may also cause independent issues, including claudication and limb ischemia. Ulnar artery disease is recognized as a common complication in scleroderma that can be shown by

Principles of management

Because there is both abnormal vasoreactivity and structural vascular disease causing RP and tissue ischemia in scleroderma, management requires a multitargeted approach. The first intervention should be to prevent vasospasm. Avoiding triggering factors, including cold environments and psychological stress, has a major impact on the disease and management of these factors needs to be emphasized to patients (discussed later). Vasodilator therapy is the first line of drug therapy and should be

Case One

A 31-year-old woman presents to clinic complaining of uncomfortable color changes of her fingers triggered by the cold. The history confirms that she has RP, with attacks since the age of 27 years that have increased in frequency and severity over the last 3 years. She has mild gastrointestinal reflux disease but no clinical or laboratory evidence of interstitial lung disease or pulmonary hypertension. She was recently diagnosed with limited scleroderma when mild sclerodactyly and a positive

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    Disclosures: The authors have no relevant disclosures.

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