ABC of arterial and vascular disease: Vasculitis

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Abstract
Large vessel vasculitisGiant cell arteritis (temporal arteritis)Clinical features include unilateral throbbing headache, facial pain, and claudication of the jaw when eating. Visual loss is a feared symptom and may be sudden and painless, affecting part or all of the visual field. Diplopia may also occur. Giant cell arteritis is the most common type of primary systemic vasculitis with an incidence of 200/million population/year.View larger version: In this window In a new window Spectrum of systemic vasculitides organised according to predominant size of vessels affected (adapted from Jennette et al, Arthritis Rheum 1994;37:187-92)Treatment is with high dose corticosteroids (40-60 mg/day), which should be started as soon as the diagnosis is suspected to avoid visual loss. The diagnosis is confirmed by biopsy of the affected artery, done within 24 hours of starting corticosteroids. The corticosteroid dose may be reduced to 10 mg/day over six months and then more slowly to a maintenance of 5-10 mg/day. Maintenance treatment may be required for two years. The disease is monitored by measuring C reactive protein concentrations, erythrocyte sedimentation rate, or plasma viscosity.Takayasu's arteritisTakayasu's arteritis is most common in Asia and the Far East and affects women more than men. Disease of the arteries supplying the arms, head, neck, and heart leads to the aortic arch syndrome with claudication of the arm, loss of arm pulses, variation in blood pressure of more than 10 mm Hg between the arms, arterial bruits, angina, aortic regurgitation, syncope, stroke, and visual disturbance. The descending aortic syndrome may cause bowel ischaemia or infarction, renovascular hypertension, and renal impairment.View larger version: In this window In a new window C reactive protein concentration (>10 mg/l) and erythrocyte sedimentation rate (>18 mm/h) are raised at time of diagnosis of giant cell arteritis but fall to normal levels after starting immunosuppressant therapy.Diagnosis is by angiography or magnetic resonance angiography. Treatment of acute disease in patients with high C reactive protein concentration or erythrocyte sedimentation rate is with corticosteroids. Cytotoxic drugs such as cyclophosphamide can be added if steroids alone do not control the disease. Surgery or angioplasty may be required for stenoses once active inflammation has been controlled.

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