Anticoagulation is unproven as chronic therapy for Raynaud's phenomenon unless patients have a secondary hypercoagulation disorder, such as antiphospholipid antibody syndrome.
▸ Thrombolytic therapy. There is little evidence to support the use of thrombolytic therapy in patients with scleroderma who have catastrophic Raynaud's events, and he does not recommend it.
▸ Surgical sympathectomy. Surgical sympathectomy denervates the finger. '“We use a digital sympathectomy when medical therapy is not working,” he said. However, there is conflicting evidence on the efficacy of this procedure.
In a retrospective review of 129 fingers in 38 patients with scleroderma and chronic digital ischemia, 86% reported improved symptoms with periarterial sympathectomy after a follow-up period of up to 41 months.
The study was presented at the 2003 annual meeting of the American College of Rheumatology (Arthritis Rheum. 2003; 48[suppl. 9]:S560).
However, a separate systematic review of 251 digits came to less optimistic conclusions. It found that 14% of all patients required amputation and 18% of patients had ulcer recurrence, after up to nearly 5 years of follow-up.
“Some type of postoperative complication was reported in 37% of patients with systemic sclerosis,” according to the study's authors, who noted that the literature is marred by a number of shortcomings (J. Rheumatol. 2003;30:1788–92).
“Sympathectomy can reverse an ischemic crisis, but may not cure the problem,” said Dr. Wigley. It sometimes leads to finger contractures and fibrosis, “so I don't use it lightly,” he added.
“Usually it gets the patient out of a crisis. A year later the Raynaud's is back, but it tends not to be as severe.”
▸ Vascular surgery. A retrospective case series reported on the outcomes of revascularization of occluded ulnar arteries in eight patients with scleroderma and severe Raynaud's phenomenon with digital ulcers.
The patients had a positive Allen's tests, had angiograms showing occlusive disease, and had failed to improve with nonoperative treatment.
All eight patients “experienced dramatic improvement in Raynaud's phenomenon and refractory digital ulcers,” according to the authors (J. Rheumatol. 2002;29:102–6).
In isolated cases, “you need to consider it,” said Dr. Wigley. However, he advises caution. “You have to be dealing with an experienced vascular surgeon and be very conservative about attacking these blood vessels. … Patients have lost not just their finger but parts of their hands.”
Dr. Wigley said it's rare to find a lesion that is an appropriate target for microsurgical repair. “Most of the time you have the combination of a large vessel, such as an ulnar occlusion, and a microvascular disease, so there's nothing you can connect to, to make a repair.”
Preventing Recurrences
For prevention of recurrent crises, “there's no drug more effective than warm temperatures,” said Dr. Wigley
When cold is unavoidable, Dr. Wigley recommends using continued vasodilator therapy, aspirin, and antioxidants. Statins “will play a bigger role as we learn more about these agents.”
Other options for preventing recurrences include:
▸ Iloprost infusions. Recent trials from Europe suggest that prophylactic infusions of iloprost may reduce the frequency and severity of Raynaud's events among scleroderma patients (Med. Clin. [Barc.] 2004;122:501–4; Clin. Rheumatol. 2002;21:244–50).
▸ Bosentan. Findings from a recent trial demonstrated a reduction in digital ulcers among scleroderma patients after treatment with the endothelin receptor antagonist bosentan (Arthritis Rheum. 2004; 50:3985–93).