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Part II
• Disorders
Raynaud’s Disease
R
aynaud’s disease is one of several primary disorders char-
acterized by episodic spasms of the small peripheral arter-
ies and arterioles, precipitated by exposure to cold or stress.
This condition occurs bilaterally and usually affects the hands
or, less often, the feet. It’s benign, requires no specific treat-
ment, and has no serious sequelae. Raynaud’s
phenomenon
, how-
ever, is secondary to any of several connective disorders — such
as scleroderma, systemic lupus erythematosus, or polymyosi-
tis — and progresses to ischemia, gangrene, and amputation.
Distinguishing between the two disorders is difficult because
some patients experience mild symptoms of Raynaud’s disease
for several years and then develop overt connective tissue dis-
ease, especially scleroderma.
Age Alert
Raynaud’s disease is most prevalent in females,
particularly between puberty and age 40.
Causes
Raynaud’s Disease
•
Unknown; family history is a risk factor
Raynaud’s Phenomenon
•
Connective tissue disorders, such as scleroderma, rheuma-
toid arthritis, systemic lupus erythematosus, or polymyositis
•
Pulmonary hypertension
•
Thoracic outlet syndrome
•
Arterial occlusive disease
•
Myxedema
•
Trauma
•
Serum sickness
•
Exposure to heavy metals
•
Long-term exposure to cold, vibrating machinery (such
as operating a jackhammer), or pressure to the fingertips
(as in typists and pianists)
Pathophysiology
Raynaud’s disease is a syndrome of episodic constriction of the
arterioles and arteries of the extremities, resulting in pallor
and cyanosis of the fingers and toes. Several mechanisms may
account for the reduced digital blood flow, including:
•
intrinsic vascular wall hyperactivity to cold
•
increased vasomotor tone due to sympathetic stimulation
•
antigen-antibody immune response (most likely because
abnormal immunologic test results accompany Raynaud’s
phenomenon).
Signs and Symptoms
•
Bilateral blanching (pallor) of the fingers after exposure to
cold or stress:
•
Vasoconstriction or vasospasm reduces blood flow.
•
Cyanosis caused by increased oxygen extraction results
from sluggish blood flow.
•
Spasm resolves, and fingers turn red (rubor) as blood
rushes back into the arterioles.
•
Cold and numbness
•
Throbbing, aching pain, swelling, and tingling
•
Trophic changes (as a result of ischemia), such as sclerodac-
tyly, ulcerations, or chronic paronychia
DiagnosticTest Results
•
Antinuclear antibody (ANA) titer identifies autoimmune
disease as an underlying cause of Raynaud’s phenom-
enon; further tests must be performed if the ANA titer
is positive.
•
Doppler ultrasonography shows reduced blood flow if the
symptoms result from arterial occlusive disease.
Treatment
•
Avoiding triggers, such as cold, and mechanical or chemical
injury
•
Smoking cessation and avoidance of decongestants and caf-
feine to reduce vasoconstriction
•
Calcium channel blockers, such as nifedipine, diltiazem,
and nicardipine
•
Alpha-adrenergic blockers, such as phenoxybenzamine or
reserpine
•
Biofeedback and relaxation exercises to reduce stress and
improve circulation
•
Sympathectomy or amputation
Complications
•
Ischemia
•
Gangrene
•
Amputation