Worst outcomes if missed
Permanent visual loss – early recognition and treatment is essential to prevent permanent visual loss that untreated occurs in up to 20%.
Epidemiology
- Most common form of large vessel vasculitis – but in the UK the age adjusted annual incidence rate is just 22/100,000 person-years1
- A full-time GP should expect to see one new case every two years2
- Only affects older patients, almost all will be >50 years and more common in over 75 year olds.
- Affects women 2-3 times more commonly than men.
- More common in Caucasians.
- Overlap with polymyalgia rheumatica (PMR) - up to 50% will have co-existent PMR symptoms.
Symptoms and signs
New/abrupt onset headache – the most common presenting symptom, it is usually unilateral in the temporal area and occasionally diffuse or bilateral. Consider temporal arteritis in any patient over 50 that presents with a new onset headache.
Temporal tenderness/Scalp pain - usually localised to the temples, but may be diffuse. Ask about pain on combing hair. Patients may have enlarged, tender, non-pulsatile or beaded temporal arteries which are tender to palpate.
Visual symptoms - patients may complain of blurring of vision, diplopia or amaurosis fugax. Patients may develop an anterior ischaemic optic neuropathy. Remember that any visual disturbance in a patient with suspected temporal arteritis is an indication for steroid treatment and urgent ophthalmology review.
Jaw and tongue claudication - occurs in around 20% of patients who may describe pain on chewing, which resolves after eating.
Systemic symptoms - patients may complain of low grade fever, weight loss, loss of appetite, depression and fatigue.
Polymyalgic symptoms - up to 50% of patients may complain of polymyalgic symptoms such as bilateral shoulder and hip girdle stiffness or may have been previously diagnosed with PMR.
Elevated acute/phase response: most patients with temporal arteritis will have a raised ESR/CRP. Rarely the ESR is normal.2
Differential diagnoses
- Other causes of headache such as migraine. Don’t forget to ask about previous headaches.
- ENT pathology such as sinus, TMJ and ear disease
- Cervical spine disease - ask about neck pain or arm symptoms
- Herpes zoster
- Other causes of acute vision loss (e.g. TIA)
- Serious intracranial pathology eg base of skull lesions – although these are uncommon.
Investigations and referrals
- Investigations should include urgent ESR/CRP but do not wait for results before starting treatment.
- If temporal arteritis is suspected, urgent treatment should be started with prednisolone 40-60mg daily to prevent visual loss.3,4
- If there are no contraindications, low dose aspirin 75mg/day should also be started to reduce complications such as stroke, together with a PPI and bone protection (weekly bisphosphonate and calcium/vitamin D supplementation).3,4
- Patients should be referred urgently according to local arrangements to either ophthalmology (especially if any visual symptoms) or rheumatology, for confirmation of diagnosis and so further tests such as temporal artery ultrasound and biopsy can be undertaken.
Five key questions
1. Does the patient have a new onset headache? - headache is the commonest presenting feature
2. Does the patient describe any scalp or temporal tenderness?
3. Is there any pain on chewing? - both of these features indicate a higher risk of ophthalmic complications.
4. Does the patient have any visual problems? - any visual symptoms is an indication for urgent ophthalmology review
5. How old is the patient? - temporal arteritis generally occurs in patients over 50 years.
Five red herrings
1. Absence of headache - occasionally headache is not a prominent feature in patients with other features eg scalp tenderness and jaw claudication
2. Patients may not describe other features such as jaw claudication or temporal tenderness-absence of these symptoms does not exclude the diagnosis.
3. Normal ESR/CRP-although uncommon this does not exclude the diagnosis.
4. Temporal artery biopsy may be negative due to presence of skip lesions.
5. Remember that many older people may have a slightly elevated ESR - a rule of thumb is that the ESR should be age (years) divided by two.
Dr Samantha Hider is a consultant rheumatologist at the Haywood Hospital, Stoke-on-Trent, and senior lecturer at Keele University
References
1. Smeeth L, Cook C, Hall AJ. (2006) Incidence of diagnosed polymyalgia rheumatica and temporal arteritis in the United Kingdom, 1990-2001. Annals of the rheumatic disease, 65(8) 1093-1098.
2. Barraclough K. Mallen CD. Helliwell T. Hider SL. Bhaskar D. (2012) Diagnosis and management of giant cell arteritis. British Journal of General Practice, 62 (599); 329-330.
3. Royal College of Physicians. (2010) Diagnosis and management of giant cell arteritis. Giant Cell Arteritis Guideline Development Group. 10(4):381-6.
4. Dasgupta B, Borg FA, Hassan N, Alexander L, Barraclough K, Bourke B, Fulcher J, Hollywood J, Hutchings A, James P, Kyle V, Nott J, Power M, Samanta A. (2010) BSR and BHPR Standards, Guidelines and Audit Working Group. BSR and BHPR guidelines for the management of giant cell arteritis. Rheumatology (Oxford). 49(8); 1594-7.
