In station 5 you may be asked to assess an elderly patient who has a headache with or without blurred vision.
HISTORY TAKING (3 minutes)
- SOCRATES and headache RED FLAGS
- TIMING- since when, all the time/come and go, progression, sudden/gradual onset, new headache?
- GCA specific questions:
- Age (if not already known)
- Change in vision
- Blurring/loss of vision/double vision
- 1 or both eyes
- Scalp pain and tenderness: does it hurt when you brush or comb your hair/rest head on pillow/wash hair?
- Jaw/tongue claudication: are you managing to eat ok? Can you chew your food? Does it hurt when you chew your food? Any tongue pain?
- PMR symptoms: any difficulty turning in bed/getting out of bed or chair/climbing stairs? Shoulder pain and stiffness e.g. when lifting arms?
- Systemic symptoms: Fever, night sweats, weight loss, anorexia, myalgia, fatigue
- Arm pains (limb claudication)
- Chest pain
- PMH, Dx, Fx, Sx
EXAMINE (3 minutes)
- Temporal arteries- tender/thickened/beaded/reduced or absent pulsation
- Scalp tenderness
- Ask to raise arms- any pain/stiffness
- Examine eyes
- VA, eye movements, visual fields, RAPD, *fundoscopy* pale swollen optic disc with haemorrhages (anterior ischaemic optic neuritis)
If time allows…..
- Feel pulses- carotids, brachial, radial (may be asymmetry)
- Offer left and right arm BPs (may be asymmetry)
- Listen for bruits- carotids, axillary, brachial, femoral
- Cranial Nerves- 5,7
- Quickly test power, sensation
ICE + EXPLANATION (2 minutes)
A suggested explanation is as follows:
“There are lots of different causes for headache and blurred vision but given what you’ve told me I want to test for a condition called giant cell arteritis/temporal arteritis- have you heard of it? It is caused by inflammation of arteries usually in the head and neck. ‘Giant cell’ just means big cells which are seen in the walls of the arteries when we look at a tissue sample of the artery. It is a cause of headache in patients over 50 years old.
It is important that we start treatment straight away with steroid tablets to reduce the inflammation because untreated this condition can cause the blood supply to the eye to become blocked causing blindness or cause a stroke by blocking an artery in the brain. The chances of this happening is much reduced by starting steroid tablets immediately.
Today I want to arrange for you to have some blood tests to look for inflammation and I will book for you to have a biopsy (tissue sample) of your temporal artery within the next few days to help confirm the diagnosis. This is done under local anaesthetic.
In mean time I want you to take 40mg prednisolone each day which should help alleviate your headache. We start with a high dose and then reduce the dose over weeks. It’s important that you don’t stop steroids suddenly, avoid NSAIDS, increase the dose when ill. There are some side effects of steroid tablets but the benefit outweighs the risk. I will give you a tablet to protect your stomach.
I’m also going to refer you to see a rheumatologist as this is their area of expertise and an ophthalmologist today about your vision.
You shouldn’t drive until told it’s safe to do so
You will be seen in clinic with the results of the biopsy and blood tests”
Visual loss occurs in up to 20%. Neuro-ophthalmic features typical of GCA include anterior ischaemic optic neuritis and central retinal artery occlusion. The headache is usually new unilateral abrupt onset in the temporal area. Occasionally it can be diffuse or bilateral.
Predictive features of neuro-opthalmic complications are: jaw claudication, diplopia and temporal artery abnormalities
The American College of Rheumatology classification criteria for giant cell arteritis:
For purposes of classification, a patient shall be said to have giant cell (temporal) arteritis if at least three of these five criteria are present.
1 Age at disease onset 50 years
2 New headache
3 Temporal artery abnormality
4 Elevated erythrocyte sedimentation rate 50 mm/hr
5 Abnormal artery biopsy: biopsy specimen showing vasculitis characterised by a predominance of mononuclear cell infiltration or granulomatous inflammation, usually with multinucleated giant cells
What blood tests will you do?
CRP, ESR, PV, FBC (low Hb and raised platelets), ALP, U+E, LFT. (NB: inflammatory markers can be normal)
- Herpes zoster
- Migraine or other causes of headaches
- Serious intracranial pathology e.g. infiltrative retro-orbital or base of skull lesions
- Other causes of acute vision loss e.g. TIA
- Cervical spine disease
- ENT pathology e.g. sinus, TMJ and ear disease
- GCA is a medical emergency!
- High dose prednisolone immediately on suspicion! This can be sight-saving!
- Visual loss occurs early and, once established, rarely improves
- 40mg if uncomplicated GCA (no jaw claudication or visual symptoms)
- 60mg if complicated (jaw claudication or visual symptoms)
- If visual loss/amaurosis fugax/evolving visual loss: get urgent ophthalmological opinion and give iv methylprednisolone 500mg – 1g daily for 3 days before oral steroids to save vision in the other eye. If one eye is affected there is high risk (20–50%) of bilateral vision loss with delay or stoppage of treatment
- Don’t forget bone protection (bisphosphonate an calcium/vitamin D) and proton pump inhibitor
- Request a temporal artery biopsy: TAB can remain positive for 2-6 weeks after starting steroids. TAB may be negative in some patients with GCA, due to the presence of skip lesions or to sub-optimal biopsy. Even if the TAB is negative, a patient should be regarded as having GCA if there is a typical clinical and laboratory picture and response to steroids. A temporal artery ultrasound may be helpful in this situation ( see a hypoechoic halo, occlusions and stenosis)
- The symptoms should respond rapidly to steroid followed by resolution of the inflammatory response.
- Low dose aspirin (75mg) should be considered for GCA patients if there are no contraindications
- PET/MRI for suspected large vessel involvement e.g. prominent systemic symptoms, limb claudication, persistently high inflammatory markers despite steroids
What will rheumatologists monitor in clinic?
Symptoms of GCA, PMR and large vessel vasculitis, inflammatory markers, steroid side effects including glucose, osteoporosis. They will taper steroid dose in the absence of clinical symptoms, signs and laboratory abnormalities suggestive of active disease. They will observe for relapse. For recurrent relapse, methotrexate may be used as an adjuvant therapy.
Written by Dr Sarah Kennedy
Resources used to write this document include those listed in the references section of this webpage and also:
BSR and BHPR guidelines for the management of giant cell arteritis