Laboratory studies

1. A complete blood count with differential, urinalysis, and tests for ESR, ANA, and RF can help identify an underlying systemic disorder in the initial evaluation. Additional tests should be ordered accordingly. Evaluation for the presence of a hypercoagulable state, cryoglobulins, hypothyroidism, and anti-centromere and anti-topoisomerase antibodies may be necessary. Chest radiography might reveal a bony cervical rib or basal lung fibrosis. Echocardiography is useful for exclusion of thromboemboli or septic emboli, and nerve conduction studies for nerve compression syndromes.

2. Vascular laboratory evaluation may include a Doppler study of vessels to assess the patency of the superficial palmar arch and proximal larger arteries. Additionally, finger photoplethysmography and finger blood pressure determination (with a pneumatic cuff placed around the proximal phalanx and a photoplethysmograph placed over the distal phalanx), performed at a warm ambient temperature to avoid vasoconstriction, may prove very useful. Abnormal photoplethysmographic waveforms and brachial-finger pressure gradients of more than 20 mm Hg indicate fixed arterial obstruction somewhere in the vascular pathway supplying the terminal phalanx.

3. Arteriography (or magnetic resonance angiography) is necessary only when a proximal embolic source is suspected or microsurgical arterial reconstruction of the superficial palmar arch is contemplated. Sympathetic blockade is recommended prior to angiograpy in order to avoid reflex vasospasm due to vascular trauma.

D. Differential diagnosis. RP, which is an episodic disorder, must be differentiated from processes characterized by persistent vasospastic ischemia. In the disorders described below, vasoconstriction is probably limited to arterioles and does not involve the digital arteries.

1. Acrocyanosis, like RP, is exacerbated by low temperatures and emotional stress. However, cyanosis is not confined to the digits but extends diffusely to the hands or feet. Its idiopathic form is a benign disorder.

2. Livedo reticularis, characterized by a persistent purple or blue mottling of the skin, predominantly involves the extremities; it is usually a benign condition. However, it may be vasculitic in nature (livedo reticularis with ulceration or atrophie blanche) or secondary to a systemic disorder such as SLE or the antiphospholipid syndrome.

III. Treatment. Adequate reassurance and patient education on how to avoid cold exposure is often all that is needed for patients with mild primary RP. Temperature biofeedback, in combination with different relaxation techniques or conditioning treatment, has also been helpful for such patients. Severe primary RP compromising quality of life because of frequent uncomfortable attacks and the constant need to practice cold avoidance will usually respond to pharmacologic therapy with calcium channel blockers. Therapy of secondary RP should address both vasospasm and the underlying disorder. Notably, vasculitis will require corticosteroids or immunosuppressive therapy. Antiphospholipid syndrome, other thrombophilic disorders, and thromboembolic events require appropriate anticoagulation therapy. Severe involvement of large vessels (secondary to trauma, atherosclerosis, or vasculitis) will require vascular surgery. Microsurgical revascularization, if available, can be effectively employed for occlusive lesions affecting the wrist arteries and superficial palmar arch. Notably, these are frequently seen in SSc.

A. General measures. Avoidance of cold temperatures and the use of warm clothing, mittens, and even electrically heated gloves cannot be overemphasized. Tobacco smoking should be discontinued. Repetitive traumatic injury to the extremities should be avoided. Beta blockers, if used, should be appropriately substituted. For acute and severe ischemic pain, which may further augment sympathetically mediated vasospasm, adequate analgesia will be necessary. Extreme care should be undertaken to prevent or treat infection (usually with Staphylococcus) of ischemic ulcers.

B. Drug therapy of vasospasm. Vasodilators are more helpful in cases in which the vasospastic component is prominent. If a significant occlusive component is present, the benefit may be less significant.

1. Calcium channel blockers are the best-studied agents and the usual first choice. Some calcium channel blockers, in addition to causing vasodilation, may inhibit in vivo platelet activation and enhance the thrombolytic activity of blood. However, they can aggravate esophageal reflux or constipation. They should be avoided during pregnancy.

a. Nifedipine can decrease the number and severity of episodes of RP. Side effects are common and include headache, tachycardia, flushing, light-headedness, and edema. The initial dosage is 10 mg orally three times daily with upward titration to the maximum tolerated dose. The slow-release preparations (30 or 60 mg once daily) are thought to have fewer side effects. Because amlodipine is very closely related to nifedipine, it is also used (5 to 10 mg daily).

b. Diltiazem, another calcium channel inhibitor, may be efficacious in patients with primary or vibration-induced RP. The dosage is 30 mg three to four times daily.

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