Clinical Review ABC of arterial and vascular disease

Vasculitis

BMJ 2000; 320 doi: http://dx.doi.org/10.1136/bmj.320.7245.1325 (Published 13 May 2000) Cite this as: BMJ 2000;320:1325
  1. C O S Savage,
  2. L Harper,
  3. P Cockwell,
  4. D Adu,
  5. 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.

    Temporal artery biopsy specimen with giant cell inflammation.

    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.

    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.

    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

    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.

    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 …

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