This disease, involving the small vessels of the lower limbs, occurs in young men who smoke. It is thought by some workers to be indistinguishable from atheromatous disease. However, pathologically there is inflammation of the vessels that may indicate a separate disease activity. Clinically it presents with peripheral ischaemia and patients must stop smoking.
This is rare, except in Japan. It is known as ‘pulseless disease’ or the aortic arch syndrome. It is of unknown aetiology and occurs in young females. There is a vasculitis involving the aortic arch as well as other major arteries. There is also a systemic illness, with pain and tenderness over the affected arteries. Absent peripheral pulses and hypertension are usually found. Corticosteroids help the constitutional symptoms. Heart failure and cerebrovascular accidents eventually occur, but most patients survive for at least 5 years. Kawasaki disease (mucocutaneous lymph node syndrome)
This is an uncommon acute febrile illness of early childhood. There is a generalized vasculitis with involvement of the coronary arteries and lymphadenopathy.
This gives rise to:
• Uncomplicated aortitis
• Aortic aneurysms, usually in the ascending part
• Aortic valvulitis with regurgitation
• Stenosis of the coronary ostia
The diagnosis is confirmed by serology. Treatment is with penicillin. Aneurysms and valvular disease are treated as necessary by the usual methods.
Connective tissue disorders
These cause vasculitis and can’ give rise to peripheral vascular disease.
Raynaud’s disease and phenomenon
Raynaud’s phenomenon consists of spasm of the arteries supplying the fingers or toes and is usually precipitated by cold and relieved by heat. When Raynaud’s phenomenon occurs without any underlying disorder, it is then known as Raynaud’s disease. This is a common disease affecting 5% of the population and occurring predominantly in young women. The disorder is usually bilateral and fingers are affected more commonly than toes. There is an initial pallor of the skin resulting from vasoconstricion and this is followed by cyanosis due to sluggish blood flow. Redness finally occurs owing to hyperaemenia. The duration of the attacks can be variable and can sometimes last for hours. Numbness and burning of the fingers usually ally occurs and pain can be severe, particularlv in the rewarming phase.
Between the attacks the pulses and the digits appear normal, but trophic changes with small areas of gangrene can occur in severe and persistent cases.
Primary Raynaud’s disease must be differentiated from secondary causes of Raynaud’s phenomenon,which are chiefly disorders of connective tissue, particularly systemic sclerosis. It can also occur in cryoglobulinaemia and as a side-effect of drug treatment, especiallv with B blocking agents.
No treatment is usually required for the attacks but any underlying disease must be looked for.The hand and feet should be kept warm, and smoking should be avoided. f3-Blockers should be stopped. Nifedipine 10 mg three times daily may be helpful.
Varicose veins are a common problem, sometimes giving rise to pain. They are treated by injection or surgery. Venous thrombosis Thrombosis can occur in any vein, but the veins of the leg and the pelvis are the commonest sites.
This commonly involves the saphenous veins and is often associated with varicosities. Occasionally the axillary vein is involved, usually as a result of trauma. There is local superficial inflammation of the vein wall, with secondary thrombosis.
The clinical picture is of a painful, tender, cord-like structure with associated redness and swelling. The condition usually responds to symptomatic treatment with rest, elevation of the limb and analgesics (e.g. non-steroidal anti-inflammatory drugs). Anticoagulants are not necessary, as embolism does not occur from superficial thrombophlebitis.
A thrombus forms in the vein, and any inflammation of the vein wall is secondary to this. Thrombosis commonly occurs after periods of immobilization, but it can occur in normal individuals for no obvious reasons. The precipitating factors are discussed.
Deep-vein thrombosis occurs in 50% of patients after prostatectomy or following a cerebral vascular accident. In addition, one-third of patients with a myocardial infarct have a deep-vein thrombosis.
Thrombosis can occur in any vein of the leg, but is particularly found in veins of the calf. It is often undetected; autopsy figures give an incidence of over 60% in hospitalized patients.
CLINICAL FEATURES. The major presenting features are:
ASYMPTOMATIC, presenting with clinical features of pulmonary embolism.
PAIN IN THE CALF, often presenting with swelling, redness and engorged superficial veins. The affected calf is often warmer and there may be ankle oedema. Homan’s sign (pain in the calf on dorsiflexion of the foot) is often present, but is not diagnostic and occurs with all lesions of the calf.
Thrombosis in the iliofemoral region can present with severe pain, but there are often few physical. signs apart from occasional swelling of the thigh and/or ankle oedema.
Complete occlusion, particularly of a large vein, can lead to a cyanotic discoloration of the limb and severe zedema, which can very rarely lead to venous gangrene. Pulmonary embolism can occur with any deep-vein thrombosis but is more frequent from an iliofemoral thrombosis and is rare with thrombosis confined to veins below the knee. Spread of thrombosis can occur proximally without clinical evidence, so the extent of the thrombosis must be carefully assessed.
INVESTIGATION. Clinical diagnosis is unreliable and confirmation of an iliofemoral thrombosis can usually be made with ultrasound or Doppler ultrasound. Belowknee thromboses can only reliably be detected by venography but whether this is necessary is questionable (see Treatment). A venogram is performed by injecting a vein in the foot with contrast which will detect virtually all thrombi that are present.
TREATMENT. The main aim of therapy is to prevent pulmonary embolism and all patients with thrombi above the knee must be anticoagulated. Anticoagulation of below-knee thrombi is controversial. Bed rest is advised until the patient is fully anticoagulated. The patient should then be mobilized, with an elastic stocking giving graduated pressure over the leg. Heparin is given normally for 48 hours but how long warfarin should be given is debatable-3 months is the usual recommended period but 4 weeks is long enough if a definite risk factor (e.g. bed rest) has been present.
The INR should be at 2-3.0. Anticoagulants do not affect the thrombus that is already present. Thrombolytic therapy is occasionally used for patients with a large iliofemoral thrombosis.
PROGNOSIS. Destruction of the deep-vein valves produces a clinically painful, swollen limb that is made worse by standing and is accompanied by oedema and sometimes venous eczema. It occurs in approximately half of the patients with a clinically symptomatic deep-vein thrombosis, and it means that elastic support stockings are then required for life.
PREVENTION. Subcutaneous low-dose heparin should be given to patients with cardiac failure, a myocardial infarct or surgery to the leg or pelvis. Early ambulation is indicated as most thromboses occur within the first 72 hours following surgery. Leg exercises should be encouraged and patients should not sit in a chair with their legs immobilized on a stool. An elastic support stocking should be given to patients at high risk, e.g those with a history of thrombosis or with obesity.
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