ABC of arterial and vascular disease Vasculitis

COS Savage, L Harper, P Cockwell, D Adu, A J Howie

Vasculitis is inflammation of blood vessel walls. The clinical and pathological features are variable and depend on the site and type of blood vessels that are affected. Diseases in which vasculitis is a primary process are called primary systemic vasculitides.

The main types of vasculitides can be described using clinical features and pathological findings according to the Chapel Hill Consensus Conference. These names and definitions will be followed in this article. Definitive classification of systemic vasculitis is unsatisfactory since aetiology and pathogenesis are rarely known, and clinical and histological features overlap. Vasculitis may also occur as a secondary feature in other diseases, such as systemic lupus erythematosus and rheumatoid arthritis.

Fever, night sweats, malaise, myalgia, and arthralgia are common in all types of vasculitis. Active vasculitis is usually associated with an acute phase response with an increase in C reactive protein concentration, erythrocyte sedimentation rate, or plasma viscosity.

Large vessel vasculitis

Temporal Artery Biopsy

Temporal artery biopsy specimen with giant cell inflammation

Giant 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.

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 arteritis

Takayasu'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.

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.

Temporal artery biopsy specimen with giant cell inflammation

Definitions of large vessel vasculitis Giant cell arteritis (temporal arteritis)

• Granulomatous arteritis of aorta and its major branches, especially extracranial branches of carotid artery

• Often affects temporal artery

• Usually occurs in patients older than 50 years

• Often associated with polymyalgia rheumatica Takayasu's arteritis

• Granulomatous inflammation of aorta and its major branches

• Usually occurs in patients younger than 50 years

Aorta (large artery)

Renal artery (medium sized artery)

Interlobular artery (small artery)

Arteriole

Chapel Hill Vasculitis Classification

Giant cell (temporal) arteritis and Takayasu's arteritis

Polyarteritis nodosa and Kawasaki disease

Glomerulus

I Microscopic polyangiitis, Wegener's granulomatosis and | Churg-Strauss syndrome

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