Raynaud Phenomenon
GENERAL PRINCIPLES
Raynaud phenomenon (RP) is a vasospasm of the digital arteries and can result in ischemia of the digits. It manifests as repeated episodes of color changes in the finger, toes, and less commonly ears and nose after cold exposure or emotional stress.
The sequential color changes are white to blue to red, but this triad may not be seen in all patients. Well-demarcated pallor (white phase) is the most definitive phase. Patients may also experience numbness during the ischemic phase and pain during the rewarming phase.Classification
• RP can be classified as primary (idiopathic) or secondary if an underlying condition is present. Secondary RP is associated with multiple conditions and can be divided into different categories.
î Systemic rheumatic disease: systemic sclerosis, MCTD, SLE, inflammatory myopathies, Sjogren syndrome, Burger disease, RA, vasculitis
î Drugs or chemical induced: ergots, beta-blockers, bromocriptine, interferon-alfa, chemotherapeutic agents (vinblastine, bleomycin, cisplatin), vinyl chloride
î Traumatic: pneumatic hammer operators, rock drillers, lumberjacks, etc.
î Hyperviscosity syndrome: myeloproliferative disorders, paraproteinemia, leukemia, cryoglobulinemia, and cold agglutinins
î Occlusive arterial disease: thrombotic/embolic arterial occlusion, thoracic outlet syndrome
î Endocrinopathies: hypothyroidism, pheochromocytoma, carcinoid
î Other: infections, complex regional pain syndrome, peripheral arteriovenous fistula
• A thorough review of systems and physical examination is critical to differentiate between primary and secondary RP. Some of the other differences include:
î Primary RP is more common in women and onset is typically between the ages of 15-30 years. These patients do not develop complications like ulceration, pitting, or gangrene. Nailfold capillary examination is normal and serologies are negative (although up to one-third may have low titer positive ANA).
î Secondary RP may affect men or women. Onset tends to be in the third or fourth decade. Review of systems and physical examination may reveal signs or symptoms of an underlying condition. These patients are more prone to develop complications, nailfold capillary examination is abnormal, and serologies may be positive.
DIAGNOSIS
• Nailfold capillary microscopy (NCM) can be done by placing a drop of surgical lubricant on the cuticle of the finger and visualizing the capillaries with an ophthalmoscope set at 40 diopters.
• Abnormal NCM may show tortuous capillary loops, microhemorrhages, and avascular areas (“dropout”). No laboratory test is pathognomonic of RP. In patients with clinical suspicion of an underlying disease, studies to evaluate for CTD (ANA, extractable nuclear antigens, ACA), hypothyroidism (thyroid stimulating hormone), hypercoagulable state (CBC, serum protein electrophoresis, urine protein electrophoresis, free light chains), or cryoglobulinemia should be considered.
TREATMENT
Preventive Measures
Patients should be instructed to avoid exposure to cold, protect the hands and feet from trauma, limit caffeine intake, and discontinue cigarette smoking.
Medications
• Calcium channel antagonists (of the dihydropyridine group) are the preferred initial agents, although they may exacerbate gastroesophageal reflux and constipation.
• Alternative vasodilators such as prazosin are occasionally helpful but can have limiting side effects, including orthostatic hypotension.
• Other agents that might improve vasospasm include topical nitroglycerin applied to the dorsum of the hands.
• Phosphodiesterase inhibitor (e.g., sildenafil) and endothelin receptor antagonist (e.g., bosentan) can be used for secondary RP and are effective at controlling symptoms and prevention of ulcer formation.
• Daily low-dose aspirin therapy is often prescribed for its antiplatelet effects.
• Patients with severe ischemic digits should be hospitalized, and conditions such as macrovascular disease, vasculitis, or a hypercoagulable state should be ruled out. An IV infusion of a prostaglandin or prostaglandin analog may be considered.
Surgical Management
• Sympathetic ganglion blockade with a long-acting anesthetic agent may be useful when a patient has progressive digital ulceration that fails to improve with medical therapy.
• Surgical digital sympathectomy may be beneficial.