Definition
A.Upper extremity arterial disease is the obstruction of blood flow in the large and/or small arterial vessels of the upper extremity arteries.
Incidence
A.Upper extremity arterial disease is relatively rare. It accounts for less than 5% of patients presenting with limb ischemia.
Pathogenesis
A.Arterial vasospasm: Raynaud’s phenomenon, ergotism, vinyl chloride exposure.
B.Arterial obstruction.
1.Atherosclerosis (main cause of upper extremity arterial disease).
2.Thoracic outlet compression.
3.Embolic (e.g., cardiac or thoracic outlet in origin), aneurysms.
4.Arteritis (e.g., Takayasu arteritis or giant cell arteritis).
5.Fibromuscular disease.
6.Hypersensitivity angiitis.
7.Iatrogenic, cold, or vibration injury.
8.Dialysis steal syndrome.
9.Connective tissue disease (e.g., scleroderma, rheumatoid arthritis, systemic lupus).
10.Myeloproliferative disorders and hypercoagulable states.
11.Infection from injection of drugs and arterial procedures.
C.Bilateral symptoms may be from a systemic cause such as a connective tissue disorder.
D.Unilateral symptoms may be from a discrete occlusive lesion.
Predisposing Factors
A.Dependent on pathogenesis.
B.Patients who present with upper extremity ischemia range from young adults with nonatherosclerotic causes to elderly patients with atherosclerosis.
C.Risk factors include smoking, hypercholesterolemia, hypertension, diabetes, and age.
Subjective Data
A.Common complaints/symptoms.
1.Most patients with upper extremity arterial disease are asymptomatic; the condition is only detected by finding asymmetric arm blood pressures.
B.Common/typical scenario.
1.Raynaud’s phenomenon: Predictable sequence of color changes in finger and/or hand.
a.Pallor (white), followed by cyanosis (blue) and then rubor (red).
b.Often associated with finger numbness.
c.Pain is generally not severe, unless ulceration is present.
d.Symptoms are activated by exposure to cold and emotional stimuli.
2.Arm intermittent claudication is an unusual presentation of arm ischemia due to excellent collateral blood flow around the shoulder. However, active adults, particularly manual laborers, may experience arm claudication from subclavian or brachial artery stenosis.
3.Dizziness, or even syncope, during arm exertion may be from subclavian steal syndrome.
4.Patients with chronic upper extremity ischemia may complain of change in sensation, hand temperature, and muscle pain with use.
5.Tissue necrosis includes gangrene and poorly healing ulcerations of the fingers. (Patients may dismiss small ulcers caused by microemboli as inconsequential bruises or sores.)
6.Acute limb ischemia is covered previously.
C.Family and social history.
1.Elicit onset, duration, location,and intensity; aggravates and relieves pain.
2.Inquire about signs and symptoms of connective tissue disease such as dry eyes, difficulty swallowing, dry mouth, and arthritis.
3.Question the patient about any history of trauma, including upper extremity access for peripheral or coronary catheterization.
4.Inquire about occupational and recreational history regarding exposure to vibrating tools or toxins, as well as repetitive trauma.
5.Question the patient about cardiovascular-related conditions: Coronary artery disease, myocardial infarction, carotid artery disease, transient ischemic attack, or strokes.
6.Question the patient about other medical conditions such as diabetes, chronic kidney disease, heart failure, chronic obstructive pulmonary disease, or hematology conditions.
7.Inquire about musculoskeletal conditions such as osteoarthritis or rotator cuff injury.
D.Review of systems.
1.Musculoskeletal: Ask about arm pain with movement and at rest; ask about any swelling.
2.Dermatology: Ask about ulceration of fingers or discoloration.
Physical Examination
A.Take blood pressure in both arms.
1.10 mmHg or more difference suggests a hemodynamically significant innominate, subclavian, or axillary artery stenosis.
2.In cases of suspected claudication, arm blood pressure, should be measured at rest and after 2 to 5 minutes of exercise.
B.Inspect.
1.Hands and fingers and note temperature, color, capillary refill, ulcers, and any other lesions.
2.Fingers for clubbing, which is associated with chronic pulmonary disease. (Patients with clubbing and cold fingers may have low arterial oxygen levels as the basis for their complaint).
3.Fingers for telangiectasia and sclerodactyly, which is commonly seen with advanced scleroderma as well as other connective tissue diseases.
4.Check for splinter hemorrhages in the nail beds, which is seen with emboli.
C.Auscultate.
1.Heart to listen for arrhythmias, gallops, and murmurs.
2.Supraclavicular and infraclavicular fossa to listen for bruits which may indicate a possible subclavian artery stenosis. A supraclavicular pulsatile mass is associated with a subclavian aneurysm or cervical rib.
D.Palpate.
1.Upper extremity pulses.
a.Axillary and proximal brachial artery: The upper medial arm in the groove between the biceps and triceps muscles.
b.Brachial artery: The antecubital fossa just medial to the biceps tendon.
c.Radial artery: The wrist over the distal radius.
d.Ulnar artery: The wrist over the distal ulna.
2.Carotid, abdominal aorta, femoral, popliteal, dorsalis pedis, post tibial pulses.
E.Handheld Doppler.
1.Assess upper extremity pulses including digital pulses and note if the pulse is monophasic, biphasic, or triphasic.
F.Neurological examination, including muscle mass, muscle strength, and sensation to assess for compression of the neurovascular bundle (see section “Thoracic Outlet Syndrome“).
G.Additional bedside examination.
1.Allen’s test is recommended if there is a difference in arm blood pressure or if there is a reduced radial or ulnar pulse.
a.Allen’s test should be conducted on both arms.
b.A positive Allen’s test suggests that there is adequate dual blood supply to the hand.
i.Elevate the hand and ask the patient to clench his or her fist for 30 seconds.
ii.Pressure is applied over the ulnar and radial arteries to occlude both of them.
iii.The hand is then opened. It should appear blanched.
iv.One artery is tested by releasing the pressure over that artery to see if the hand flushes (color should return within 5–15 seconds).
v.The other artery is then tested in a similar fashion.
2.Adson’s test and Roos test can assist in assessing for thoracic outlet syndrome (see section “Thoracic Outlet Syndrome“).
Diagnostic Tests
A.Vascular laboratory: Segmental pressure measurements of the upper extremity and finger pressure measurements and waveforms.
B.Radiological imaging of arterial arm circulation such as duplex ultrasound, CT, and MRI is best reserved for vascular services as part of the treatment decision and workup.
C.Hematologic evaluation.
1.Erythrocyte sedimentation rate, C-reactive protein, antiphospholipid antibodies, antinuclear antibody titer, and rheumatoid factor to screen for underlying autoimmune disease.
2.Platelet count, since thrombocytosis can mimic Raynaud’s phenomenon.
3.Serum protein electrophoresis since serum protein abnormalities may be associated with vasospasms.
4.For patients at risk of or with suspected atherosclerotic disease, fasting lipids, fasting glucose, or serum creatinine.
Differential Diagnosis
A.Multiple etiologies; see “Pathogenesis” section.
B.Differential diagnosis includes neurogenic, musculoskeletal, and pathological causes.
Evaluation and Management Plan
A.General plan.
1.All patients with upper extremity arterial disease should have a cardiovascular risk reduction plan based on their 5-year cardiovascular risk assessment.
2.Treat underlying cause.
a.Primary Raynaud’s phenomenon.
i.Conservative management; patients advised to minimize cold exposure and stress.
b.Emboli: Manage arrhythmia and anticoagulate.
c.Connective tissue diseases: Management of disease process.
d.Occupational and recreational factors: Advise patients to minimize exposure.
3.Supportive measures.
a.Wound management.
b.Antibiotic if underlying cellulitis or wound infection (consider Flucloxacillin).
c.Pain management.
4.Vascular treatment may include:
a.Endovascular revascularization.
b.Bypass surgery.
c.Digit or limb amputation.
B.Patient/family teaching points.
1.Patients should seek out a provider if they experience any pain, numbness, tingling, weakness, or significant temperature change in their extremities.
2.Patients should also report open sores that do not heal.
C.Pharmacotherapy.
1.Frequent or severe symptoms.
a.Nifidipine 30 to 180 mg/d or amiodipine 5 to 20 mg/d.
2.Start with lowest dose and gradually increase, if needed, depending upon the response.
D.Discharge instructions (if standard accepted guidelines exist, please use discharge template).
1.Make healthy dietary changes.
2.Keep cholesterol levels down.
3.Maintain a healthy weight.
4.Smoking cessation.
Follow-Up
A.Depend on pathogenesis: Outpatient follow-up with vascular, cardiology, or rheumatology service.
B.Three-month primary care review of cardiovascular risk management.
Consultation/Referral
A.Consultation and referral is dependent on pathogenesis and severity of symptoms.
1.If clinical presentation is suggestive of large vessel disease, refer to vascular service.
2.If hematological screening is positive for autoimmune disease, refer to rheumatology service.
3.Acute limb ischemia for urgent hospital admission (see “Acute Limb Ischemia” section).
4.Necrosis and gangrene. For hospital admission, seek a vascular consult.
Special/Geriatric Considerations