Raynaud Phenomenon

Raynaud phenomenon (RP) is a syndrome of paroxysmal digital ischemia, most commonly caused by an exaggerated response of digital arterioles to cold or emotional stress.

The initial phase of RP, mediated by excessive vasoconstriction, consists of well-demarcated digital pallor or cyanosis; the subsequent (recovery) phase of RP, caused by vasodilation, leads to intense hyperemia and rubor.

Although RP chiefly affects fingers, it can also affect toes and other acral areas such as the nose and ears.

RP is classified as primary (idiopathic or Raynaud disease) or secondary.

Nearly one-third of the population reports being “sensitive to the cold” but does not experience the paroxysms of digital pallor, cyanosis, and erythema characteristic of RP.

Primary RP occurs in 2–6% of adults, is especially common in young women, and poses more of a nuisance than a threat to good health.

In contrast, secondary RP is less common, is chiefly associated with rheumatic diseases (especially systemic sclerosis) and can be severe enough to cause digital ulceration or gangrene.

Diagnostic Essentials

• Paroxysmal bilateral digital pallor and cyanosis followed by rubor.
• Precipitated by cold or emotional stress; relieved by warmth.
• Primary form: benign course; usually affects young women.
• Secondary form: more severe, sometimes causing digital ulceration or gangrene.

Clinical Findings

In early attacks of RP, only one or two fingertips may be affected; as it progresses, all fingers down to the distal palm may be involved. The thumbs are rarely affected.

During recovery there may be intense rubor, throbbing, paresthesia, pain, and slight swelling.

Attacks usually terminate spontaneously or upon returning to a warm room or putting the extremity in warm water.

The patient is usually asymptomatic between attacks.

Sensory changes that often accompany vasomotor manifestations include numbness, tingling, diminished sensation, and aching pain.

Primary RP appears more commonly in women between ages 15 and 30, symmetrically involving the fingers of both hands, unlike secondary RP (which may be unilateral and may be limited to involve only one or two fingers).

It tends to be mildly progressive. Spasm becomes more frequent and prolonged.

Unlike secondary RP, primary RP does not cause digital pitting, ulceration, or gangrene.

Nailfold capillary abnormalities are among the earliest clues of secondary rather than primary RP. The nailfold capillary pattern can be visualized by placing a drop of grade B immersion oil at the patient’s cuticle and then viewing the area with an ophthalmoscope set to 40 diopters. Dropout of capillaries and dilation of the remaining capillary loops indicate a secondary form of RP, most commonly systemic sclerosis (Figure 1–5ck) (Table 1–9ck). While highly specific for secondary RP, nailfold capillary changes have a low sensitivity.

Digital pitting or ulceration or other abnormal physical findings (eg, skin tightening, loss of extremity pulse, rash, swollen joints) provides evidence of secondary RP.

Primary RP must be differentiated from the numerous causes of secondary RP (Table 20–9).

The history and examination may suggest the diagnosis of systemic sclerosis, SLE, or mixed connective tissue disease; RP is often the first manifestation of limited systemic sclerosis (CREST syndrome). The diagnosis of many of these rheumatic diseases is supported with specific serologic tests.

RP may occur in patients with the thoracic outlet syndromes. In these disorders, involvement is generally unilateral, and symptoms referable to brachial plexus compression tend to dominate the clinical picture.

Carpal tunnel syndrome should also be considered, and nerve conduction tests are appropriate in selected cases.

Differential Diagnosis

The differentiation from Buerger disease (thromboangiitis obliterans) is usually not difficult, since thromboangiitis obliterans is generally a disease of men, particularly cigarette smokers; peripheral pulses are often diminished or absent; and, when RP occurs in association with thromboangiitis obliterans, it is usually in only one or two digits.

In acrocyanosis, cyanosis of the hands is permanent and diffuse; the sharp and paroxysmal line of demarcation with pallor does not occur with acrocyanosis.

Frostbite may lead to chronic RP.

Treatment

General Measures

Patients should wear gloves or mittens whenever in temperatures that precipitate attacks.

Keeping the body warm is also a cornerstone of initial therapy. Wearing warm shirts, coats, and hats will help prevent the exaggerated vasospasm that causes RP and that is not prevented by warming only the hands.

The hands should be protected from injury at all times; wounds heal slowly, and infections are consequently hard to control. Softening and lubricating lotion to control fissured dry skin should be applied to the hands frequently.

Cigarette smoking should be stopped and sympathomimetic drugs (eg, decongestants, diet pills, and amphetamines) should be avoided.

For most patients with primary RP, general measures alone are sufficient to control symptoms. Medical or surgical therapy should be considered in patients who have severe symptoms or are experiencing tissue injury from digital ischemia.

Medications

Calcium channel blockers are first-line therapy for RP. Calcium channel blockers produce a modest benefit and are more effective in primary RP than secondary RP.

Slow-release nifedipine (30–120 mg/day orally), amlodipine (5–20 mg/day orally), felodipine, isradipine, and nisoldipine are more effective than verapamil, nicardipine, and diltiazem.

Other medications that may be effective include angiotensin II receptor blockers, topical nitrates, phosphodiesterase inhibitors (eg, sildenafil, tadalafil, and vardenafil), or SSRIs (fluoxetine).

Severe or refractory episodes of RP in which there is a threat of digital loss may require treatment with intravenous infusions of prostacyclin or prostacyclin analogs (eg, epoprostenol, iloprost, treprostinil).

Surgical Measures

Sympathectomy may be indicated when attacks have become frequent and severe, when they interfere with work and well-being, and particularly when trophic changes have developed and medical measures have failed. Digital sympathectomy may improve secondary RP.

Prognosis

Primary RP is benign and largely a nuisance for affected individuals who are exposed to cold winters or excessive air conditioning.

The prognosis of secondary RP depends on the underlying disease; unfortunately, severe pain from ulceration and gangrene is not rare with systemic sclerosis.

When to Refer

Appropriate management of patients with secondary RP often requires consultation with a rheumatologist.

When to Admit

Patients with critical digital ischemia as evidenced by severe pain and demarcation should be admitted for intensive therapy.