SOAP. – Raynaud’s Phenomenon

Raynaud’s Phenomenon

Jill C. Cash and Julie Barnes

Definition

A.Raynaud’s phenomenon (RP) is an idiopathic disease in which an exaggerated vascular response occurs in extreme circumstances (heat, cold, and stress). It is manifested by bilateral blanching of the skin that is well demarcated, discomfort in the fingers, then cyanosis, then erythema after warming the digits.

B.Cold hands and feet are very common complaints. RP involves both cutaneous color change and cool skin temperature. Although the hands are the most common area of attacks, RP also can occur in the toes, ears, nose, face, knees, and nipples.

C.A Raynaud attack typically begins in a single finger and spreads symmetrically; however, the thumb is often spared.

D.RP may be either primary or secondary. Spontaneous remission may occur with primary RP.

E.Criteria for diagnosis of primary RP:

1.Symmetric episodic attacks.

2.No evidence of peripheral vascular disease.

3.No tissue gangrene, digital pitting, or tissue injury.

4.Negative nailfold capillary examination.

5.Negative antinuclear antibody (ANA) test.

6.Normal Erythrocyte sedimentation rate (ESR).

F.Indications of secondary RP:

1.Age of onset older than 40 years.

2.Painful severe attacks with signs of ulceration/ischemia.

3.Ischemic signs/symptoms proximal to the fingers or toes.

4.Asymmetric attacks.

5.Abnormal laboratory, suggesting vascular or autoimmune disorders.

Incidence

A.3% to 20% in women.

B.3% to 14% in men.

C.3% in African Americans.

D.Wide global differences from the United States.

Pathogenesis

A.Primary RP: Occurs alone, without any other disease process. Speculated theories include digital vasospasm because of increased response of alpha 2-adrenergic receptors and a high sympathetic vascular tone.

B.Secondary RP: Symptoms occur as a secondary manifestation from other diseases or products such as autoimmune disorders (connective tissue disorders, sclerosis, Systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), vasculitis, Sjögren’s syndrome, dermatomyositis, polymyositis, etc.), atherosclerotic diseases, hematologic disorders (polycythemia, cryofibrinogenemia, etc.), metabolic/endocrine disorders (diabetes mellitus, pheochromocytoma, myxedema), neoplastic syndromes (lymphoma, leukemia, polycythemia, monoclonal/type 1 cryoglobulinemia), infections (hepatitis B, hepatitis C, mycoplasma infections), environmental exposures/neurologic changes (frostbite, vibratory injuries from use, lead exposure, vinyl chloride exposure, arsenic exposure, organic solvents [xylene, toluene, acetone, chlorinated solvents], carpel tunnel syndrome), and some medications/drugs (cyclosporines, antineoplastics, oral contraceptives, narcotics, ergot alkaloids, bromocriptine, beta-adrenergic blocking agents, and nicotine). Symptoms may be unilateral and may affect only one or two fingers. Secondary RP usually has a poorer morbidity than the primary disease.

Predisposing Factors

A.Primary RP:

1.Female.

2.Onset of symptoms after menarche (15–30 years).

3.Smoking.

4.Emotional stress.

B.Secondary RP:

1.Onset after age 40 years.

2.Male gender.

C.Family history: Multiple family members.

D.Frostbite.

E.Vascular trauma (distal ulnar artery).

F.Vibration-induced/occupation exposure (jackhammers, pneumatic drills, weed eaters).

G.Medication-associated RP (see Table 22.1).

Common Complaints

A.Paleness of the fingertips after exposure to cold temperatures, followed by redness and discomfort after warming fingers.

B.White attack: Sharp, demarcated color of skin pallor.

C.Blue attack: Cyanotic skin.

D.White or blue attack, usually lasting 15 to 20 minutes.

E.Age of onset between 15 and 30 years.

Other Signs and Symptoms

A.Paresthesias and numbness.

B.Clumsiness of the aching hand/finger.

C.Loss of pulp in pads of fingers (severe cases).

Subjective Data

A.Determine the age of onset, time, duration, and course of presenting symptoms.

B.Question the patient regarding location and symptoms, noting blanching, followed by erythema and pain after hands are warm.

C.Note frequency of attacks.

D.Review the presence of any other skin alterations that have occurred. Does patient also note skin mottling of the arms and legs? Livedo reticularis is a lilac or violet mottling or reticular pattern that occurs during a cold response.

E.Ask the patient to identify any events that precipitate occurrences and what makes symptoms worse or better:

1.Air conditioning.

2.Grocery cold/freezer food sections.

3.Cold weather.

TABLE 22.1 Drugs That Induce Raynaud’s Phenomenon

4.Cold water.

5.Emotional stress.

6.Sudden startling.

F.Identify any other symptoms that occur at the same time such as migraine headaches.

G.Review the patient’s health history for underlying disorders such as hypothyroidism and autoimmune disorders/connective tissue disease.

H.Review current or past occupations (especially those that include the use of vibratory tools).

I.Review current medications, stimulants, herbals, and over-the-counter (OTC) medications.

Physical Examination

A.Check pulse, respirations, and BP.

B.Inspect:

1.Dermal examination: Note malar/petechial rash, telangiectasias, digital pallor, or erythema. Examine for any ulceration or signs of ischemia.

2.Inspect joints for swelling or redness and overall ischemic changes.

3.Examine several fingernails using a microscope or ophthalmoscope; examine capillaries at nailfold:

a.Normal: Fine red capillaries, lined in the same direction.

b.Abnormal: Capillaries dilated, tortuous, and irregularly spaced; avoid using the index finger for evaluation.

C.Auscultate:

1.Heart

2.Lungs

D.Palpate:

1.Palpate the joints for tenderness and peripheral pulses bilaterally.

E.Neurologic examination: Sensory function:

1.Sensory discrimination (hot/cold, sharp/dull).

2.Location of sensation (proximal/distal to previous stimuli).

3.Vibratory sensation with tuning fork (distal to proximal joints).

4.Graphesthesia (draw a number or letter in the palm of the hand with a blunt object, such as a pencil, applicator stick, or pen and have the patient identify the letter/number).

Diagnostic Tests

A.There is no gold standard diagnostic test.

B.History alone is accepted as diagnostic as no office test application consistently triggers an attack. A history of at least two color changes, pallor, and cyanosis after cold exposure is adequate for the diagnosis of RP. Ask the patient to take a picture when symptoms occur and bring it into the office for evaluation.

C.The cold water challenge test is no longer recommended.

D.Tools to assess vascular response (usually not readily available):

1.Nailfold capillaroscopy.

2.Videomicroscopy.

3.Thermography.

4.Angiography.

5.Laser Doppler.

6.Direct measures of the skin temperature and local blood flow.

E.Laboratory tests:

1.ANA.

2.ESR.

3.Thyroid profile if hypothyroidism is suspected.

4.Other tests are used, depending on suspected etiology; testing should be guided by results of history and physical examination:

a.Complete blood count (CBC).

b.Chemistry profile with renal and liver function.

c.Urinalysis.

d.Rheumatoid factor (RF).

e.Complement (C3 and C4).

f.ANA.

Differential Diagnoses

A.RP (primary vs. secondary).

B.Scleroderma.

C.SLE.

D.Occupational trauma.

E.Medication induced.

F.Peripheral vascular disease.

G.Neurovascular processes (diabetes, atherosclerosis, thromboangiitis obliterans).

Plan

A.General interventions:

1.If ulcerations are present, monitor for secondary infections. Consider topical/systemic antibiotics if secondary infection. Debridement may be necessary.

2.Biofeedback and relaxation techniques are frequently used for treatment.

B.Patient teaching:

1.Stress the importance of not smoking.

2.Keep the body warm:

a.If in extreme temperatures, wear extra clothing (thermal underwear) to maintain temperature.

b.Wear mittens instead of gloves to protect hands and keep them warm.

c.Wear a hat to conserve heat.

3.If possible, stop all medications that could be inducing symptoms. Other drugs that should be avoided include the following:

a.Decongestants.

b.Herbs that contain ephedra.

c.Medications used for migraine headaches; for example, serotonin agonists such as sumatriptan, or caffeine plus ergotamine.

4.If vibrator injury is present, stop the repetitive activity that induces symptoms. Consider alternative methods of work. If unable to totally stop the activity, decrease time spent using the vibratory equipment.

5.Emotional stress can be a trigger because of the vasoconstriction of the sympathetic nervous system being triggered. Counsel the patient regarding controlling the stress in his or her life and treatment (nonpharmacological/pharmacological) options that may be beneficial.

C.Surgical therapy (only recommended for patients resistant to initial therapies):

1.Temporary sympathectomy involving a local chemical block with lidocaine or bupivacaine (without epinephrine) relieves the pain.

2.Chemical and cervical sympathectomy may be used for severe cases with digital ischemia for temporary measures.

3.Vascular reconstruction is an option.

D.Pharmaceutical therapy:

1.Therapy may be required only during the winter months.

2.Long-acting calcium channel blockers are used. Doses may be adjusted every 4 weeks or as tolerated. Monitor by side effects: Headaches, dizziness, flushing tachycardia, and edema:

a.Nifedipine 30 to 180 mg/d.

b.Amlodipine 5 to 20 mg/d.

3.Low-dose aspirin antiplatelet therapy 75 to 81 mg/d may be considered in secondary RP with a history of ischemic ulcers or other thrombotic events.

4.Vasodilators (sildenafil) and endothelin receptor antagonists (bosentan) may be useful in refractory cases with associated digital ulcers/infarcts. However, these medications are not FDA approved for this is treatment.

Follow-Up