SNOWMASS, COLO. (FRONTLINE MEDICAL NEWS) – Persistent, widespread, intense ischemic pain in the vicinity of a digital ulcer in a patient with secondary Raynaud’s phenomenon signals impending tissue infarction and gangrene, Dr. Fredrick M. Wigley warned at the Winter Rheumatology Symposium sponsored by the American College of Rheumatology.

“When you see this you’ve got a heart attack of the finger occurring. It’s a medical emergency,” emphasized Dr. Wigley, professor of medicine, associate head of rheumatology, and director of the scleroderma center at Johns Hopkins University, Baltimore.

The pain associated with this condition is severe enough that opiates are often required. Therefore, one of Dr. Wigley’s first moves in the office is often to administer a digital block for immediate pain relief. He sticks the needle into the web of the finger and infiltrates 2% lidocaine. It’s an easy procedure to do, and it often breaks the acute event.

If the digital block doesn’t succeed in terminating the ischemic event, however, then his go-to therapy is prostacyclin infusion.

“We’re very keen at our center, and at other centers around the country as well, to go right to a prostacyclin analogue. You can do that for prevention or during an acute event. Set up a peripheral line, run in prostacyclin for 3-5 days, and it has a magical effect that lasts for 10-12 weeks. It can really break an ischemic event quickly and may also have some preventive benefit. What we have available in the U.S. is epoprostenol. In can be administered on an inpatient basis, but we set it up as outpatient therapy. Patients come in for an 8-hour period for 3-5 days in a row,” the rheumatologist explained.

This is off-label therapy. Epoprostenol’s approved indication is in treating pulmonary arterial hypertension. But there is persuasive evidence of the effectiveness of epoprostenol (Flolan) and the prostacyclins available in other countries in aborting acute digital ischemic events in patients with Raynaud’s, he said.

One prostacyclin, treprostinil, is now available as Orenitram in an oral formulation approved for treatment of pulmonary arterial hypertension. Other oral prostacyclins are in the developmental pipeline. Despite their promise of much greater patient convenience, however, at this point their use in patients with acute digital critical ischemia isn’t supported by evidence.

During those multiple, daylong outpatient treatment sessions, Dr. Wigley emphasizes the importance of staying warm, resting, and avoiding stress. He also makes sure the patient is on an optimal vasodilatory medication program, the linchpin of which is a long-acting calcium channel blocker titrated to the maximum tolerated dose.

He also does special testing in search of a potential obstruction in a larger upstream vessel that might be amenable to a corrective procedure. These tests include Doppler ultrasound, the Allen’s test, magnetic resonance angiography, and/or digital subtraction x-ray.

This multiday period of prostacyclin therapy is an excellent opportunity to initiate the use of vasculoprotective medications. The ones he’s found most helpful include statins, low-dose aspirin or another antiplatelet agent, antioxidants, and in the case of a suspected early thrombotic or embolic event, 24-72 hours of low-molecular-weight or unfractionated heparin.

There is clinical trial evidence that bosentan (Tracleer) reduces the frequency of recurrent digital ulcers. Dr. Wigley rarely uses it, however, because it’s expensive and has lots of toxicity.

Surgical sympathectomy can be finger saving.

“Don’t dillydally,” urged Dr. Wigley. “If your patient is not responding to medical therapy, don’t say, ‘Come back in a few weeks, and we’ll see how you’re doing.’ You’ll lose the finger.”

Sympathectomy has several salutary effects: the procedure rips sympathetic nerve fibers from the over-sensitive blood vessels and also gets rid of fibrous material entrapping the vessels.

In a meta-analysis of studies that included 511 proximal sympathectomies in 449 patients with secondary Raynaud’s, 89% reported sustained improvement ( J. Vasc. Surg. 2011;54:273-7 ). The available data on the less morbid alternative digital sympathectomy procedure are less extensive, but the rates of complete healing and/or decrease in digital ulcers are impressive.

Remember: Avoid tunnel vision, Dr. Wigley cautioned. Always consider the likely possibility of macrovascular disease in the setting of lower-extremity digital ischemia. Measurement of the ankle-brachial pressure index is considered a mandatory part of the clinical work-up in these patients. An ankle-brachial index of less than 0.9 has 95% sensitivity for the presence of angiographically evident cardiovascular disease.

Dr. Wigley reported serving as a consultant to Novartis and United Therapeutics and receiving research grants from Kinemed, MedImmune, and CSL Behring.