Giant cell arteritis (=temporal arteritis) is a systemic medium and large vessel vasculitis with a predilection for the extracranial branches of the carotid arteries.
Presentation may include
- Headache (around 70%)
- Jaw claudication (around 40%)
- Fever (around 30%)
- Amarosis fugax (around 25%)
The American College of Rheumatology GCA diagnostic criteria gives >90% sensitivity and specificity if >/= 3 of the following are present:
- age =/> 50
- new onset localised headache
- ESR >/= 50mm/hr
- temporal tenderness or decreased temporal pulse
- characteristic biopsy.]
Roughly 50% of people with giant cell arteritis also have polymyalgia rheumatica
Almost all patients are >50 years old
Women are more frequently affected than men (3:1)
Women are more frequently affected than men (3:1)
There is an association with HLA-DR4
Diagnosis
- ESR – usually raised
- Abnormal temporal arteries on examination
- Temporal artery biopsy
- Mononuclear infiltrates or granulomas
- Normal biopsy does not exclude diagnosis as there are ‘skip lesions’
Treatment is oral steroids (start at 1mg/kg prednisolone unless visual impairment or stroke, in which case use IV methylprednisolone) Taper slowly but continue for at least 12 months.
Complications
- Irreversible blindness (up to 10%)
- Aortic aneurysms
- Aortic regurgitation
- Ischaemic strokes
- Recurrence - up to 40% relapse within 2 years.
References