• Botulinum toxin type A: Vascular surgeons report glowing results, with rapid improvement after injection of Botox at the base of the finger. But there have been no controlled trials and Dr. Wigley said that he remains skeptical, having used it in a critical situation, with a disappointing lack of acute vasodilation.
"I don't know if it really works or not. We'll have to wait for a good study," he said.
• Bosentan: In the Randomized, Double-Blind, Placebo-Controlled, Multicenter Study to Assess the Effect of Bosentan on Healing and Prevention of Ischemic Digital Ulcers in Patients With Systemic Sclerosis (RAPIDS-1) trial, patients treated with the endothelin inhibitor had a 48% decrease in new ulcers compared to placebo during a 16-week period. They also experienced significantly improved hand function, although there was no benefit in terms of healing of existing ulcers or patient-assessed Raynaud's severity (Arthritis Rheum. 2004;50:3985-93). These findings suggest bosentan may have a role in a protective strategy aimed at reducing ischemic events, according to Dr. Wigley.
• Antioxidants: Most studies have been negative.
At the Johns Hopkins scleroderma center, the management approach taken in a new patient with Raynaud’s phenomenon with digital ischemia is to first stop the aggravating environmental factors: cold, trauma, smoking, and stress.
"None of the drugs we have is more potent than warm temperatures," the rheumatologist stressed.
Dr. Wigley puts the patient on amlodipine along with aspirin at 81 mg/day, aimed at preventing vascular occlusion. If the patient remains severely ischemic despite maximum-dose amlodipine or has digital ulcers he adds a second vasodilator, typically a phosphodiesterase inhibitor such as sildenafil, which has been shown to reduce the frequency and duration of Raynaud's attacks compared to placebo in a small, double-blind, crossover, randomized trial (Circulation 2005;112:2980-5).
He said he has a low threshold for putting Raynaud's patients on statin therapy as a vascular protection strategy. Dr. Wigley said that he was favorably impressed by findings from a randomized trial in which scleroderma patients placed on atorvastatin at 40 mg/day had a significant decrease in new ulcers compared to placebo, as well as better scores on pain scales and improved endothelial markers (J. Rheumatol. 2008;35:1801-9).
Dr. Wigley declared that he receives consulting fees and/or research grants from Actelion, Amira, Kinemed, Medimmune, Novartis, Orion, Pfizer, and United Therapeutics.