SNOWMASS, COLO. - Persistent pain and nonreversible digital discoloration in a patient with Raynaud's phenomenon are indicators of critical ischemia constituting a medical emergency.
"Raynaud's patients will often say, 'My fingers are uncomfortable. I feel pins and needles.' But when they say it actually hurts, you're in trouble. Particularly if they say, 'It hurts beyond my finger, it hurts in the palm of my hand and radiates up in my arm, I have to hang my hand off the edge of the bed to get relief, it’s worse at nighttime,' then you’ve reached the point of critical ischemia and if you don't react you're going to have big trouble," Dr. Fredrick M. Wigley said at a symposium sponsored by the American College of Rheumatology.
Dr. Fredrick M. Wigley
Although pain is the key feature marking a critical ischemic event, nonreversible discoloration is another indication. Affected digits will have well-demarcated pale-blue areas, and upon pressing down and then releasing the finger, no blood reflow is seen, explained Dr. Wigley, professor of medicine and head of the scleroderma center at Johns Hopkins University, Baltimore.
In contrast, reversibility is the hallmark of uncomplicated Raynaud's. One of the most common triggers is reaching into the frozen foods section at the supermarket. But 15 minutes after rewarming, the discoloration is reversed. Uncomplicated Raynaud's involves all the digits; the thumb is less often involved than the fingers, but it is not spared.
An acute ischemic crisis requires urgent care. Dr. Wigley's management approach begins with rest and warming of the affected hand, followed quickly by a local digital block. He injects 2% lidocaine into the web at the base of the affected finger, placing the needle tip close to the digital nerve. This accomplishes two things: It brings immediate pain relief, and it lets him see whether acute vasodilation occurs in response to the injection, an encouraging finding.
If the patient isn't already on oral vasodilator therapy with a long-acting oral calcium channel blocker, he starts amlodipine immediately. In an acute ischemic crisis, Dr. Wigley resorts to low-dose epoprostenol infused into a peripheral vein at 0.5-2.0 ng/kg per minute continuously for 3 or more days. To avoid hospitalization, he allows patients to undergo the prostacyclin infusions on an outpatient basis and go home at the end of each treatment day.
Although it's not a well-studied intervention, 48 hours of anticoagulation with unfractionated heparin or low-molecular-weight heparin makes sense in a patient with acute, rapidly advancing digital ischemia who is at risk of losing a digit, he said.
Dr. Wigley disclosed that he has received consulting fees and/or research grants from Actelion, Amira, KineMed, MedImmune, Novartis, Orion, Pfizer, and United Therapeutics.