Management of acute, severe, or progressive ischemia in a patient with confirmed or suspected Raynaud phenomenon

RP: Raynaud phenomenon; CCB: calcium channel blocker; IV: intravenous; BP: blood pressure; HR: heart rate.
* Vascular assessment includes evaluation of upper extremity pulses, auscultation for bruits, bilateral upper extremity BP, Allen test, vascular imaging (typically duplex ultrasound, possibly arteriography).
¶ Patient with RP can have underlying structural vascular disease that may be related to underlying etiology of RP (eg, systemic sclerosis [scleroderma]) or due to another process (eg, atherosclerosis, embolus). Obstruction can affect the proximal (subclavian, axillary, brachial, radial, ulnar) or distal (deep or superficial palmar arch, digital vessels) vasculature.
Δ "Proximal" vascular disease involves the subclavian, axillary, brachial, radial, and ulnar arteries. "Distal" vascular disease involves the palmar arch vessels and digital vessels.
◊ Refer for inpatient vascular evaluation. Initial treatment may include systemic anticoagulation. Vascular imaging determines the location and extent of disease. Treatment depends on the identified etiology (eg, atherosclerotic disease, embolism, dissection).
§ Patients with severe RP may require hospitalization.
¥ Refer to UpToDate content on initial management of RP.
‡ Systemic anticoagulation is initiated to minimize propagation of any possible thrombus, but is discontinued once a thrombotic cause of acute ischemia is excluded. The choice of agent depends on the treatment setting (eg, inpatient, outpatient).
† IV prostaglandin therapy is often administered in the inpatient setting; however, outpatient treatment may be appropriate for patients with an established diagnosis of RP who are otherwise healthy and the outpatient infusion center has available monitoring (eg, BP, HR).
** Alternative therapies to IV prostaglandin and/or a regional block include botulinum toxin, oral prostaglandins, and analogs. However, these therapies are of uncertain benefit or lack efficacy. Refer to UpToDate content on treatment of refractory RP.