Acute Peripheral Crisis with Raynaud's Phenomenon

Robert Terkeltaub, MD


November 07, 2000


A 34-year-old woman with a history of scleroderma and Raynaud's phenomenon presents with vasospastic painful crisis in both hands. She is taking calcium channel blockers and has lost 3 fingers from prior crises. What would be appropriate therapy? How good is iloprost for this emergency?

Anastacia Maldonado, MD

Response from Robert Terkeltaub, MD

Acute peripheral ischemic crisis is a devastating clinical problem in scleroderma. Rapid and aggressive therapy is the best strategy.[1,2] Hospitalization is helpful here, so that the patient can be kept warm and away from stress, and the digits rested and well-protected from trauma. Analgesia is essential, but because opiates can vasoconstrict, local chemical sympathectomy may be useful for both pain relief and vasodilation.

Typically, lidocaine or bupivacaine (without epinephrine) is administered in a wrist or digital block and, if effective, can be readministered over 48 hours and possibly obviate the need for a surgical digital sympathectomy. Early on, the first line of vasodilator therapy should include a sustained-release calcium channel blocker such as amlodipine or nifedipine. The role of short-acting vasodilators such as phentolamine or nitroprusside is unclear. Moreover, overly aggressive vasodilator therapy may endanger major organ perfusion, or it may worsen digital ischemia by diverting blood flow to regions with vessels more capable of vasodilation.

If available to you, intravenous prostaglandins such as iloprost can be useful as a second-line for reversal of digital ischemia.[3] Antiplatelet therapy (beyond aspirin) has not been well studied in this setting but is a rational adjunctive approach. Anticoagulation or thrombolytic therapy might be of value in refractory cases,[1,2,4] but these measures have not received adequate investigation to date.


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