What is it?
GCA (Giant cell arteritis) or temporal arteritis is a well known Ophthalmology emergency. GCA is a type of vasculitis that affects medium to larger sized vessels
Who gets it?
- Older patients >75 years old
- European population
- Women are 2x more likely
- Former pregnancy is believed to be protective
- Patients who have other rheumatological conditions eg PMR
Signs and symptoms:
|Local symptoms||Systemic symptoms|
|Headache Jaw claudication Pain in tongue Painful scalp/over temporal area Persistent pain Hardening of temporal artery Pulsating artery||Can be prodromal (before onset of fulminant disease) Malaise Fever Weight loss|
|Specific ophthalmic symptoms:||Specific ophthalmic signs:|
|Transient severe vision loss Sudden in onset Double vision Eye pain Can be bilateral (rare)||Fundoscopy can show “cotton wool” spots, papilloedema.|
How do I diagnose it?
Diagnosis is mostly based on symptoms and physicians should have a low suspicion, especially in older women presenting with headache/temporal artery tenderness.
According to NICE guidelines GCA should be suspected in any patient who is at least 50 years old with any of the following:
- New onset headache
- Temporal artery abnormality (tender/thickened/reduced or absent pulsation)
- Visual disturbances: diplopia/change in colour vision
- Fundoscopic findings are not diagnostic for GCA but include oedema and ‘cotton-wool spots’
- Scalp tenderness
- Jaw claudication
- Systemic features eg neurological symptoms (TIA/neuropathies); URTI symptoms; other vascular symptoms eg bruits/dissection
To confirm the disease standard practice is bloods, temporal artery biopsy and ultrasound imaging. Although temporal artery biopsy is now performed less commonly as it is invasive and a negative result does not rule out GCA.
- ESR (although not always elevated)
- Duplex ultrasound: swelling around vessel and non-compressible artery.
- Fluorescein angiography: filling defects observed
- MRI/PET scans – not routinely used
Biopsy: temporal artery biopsy (can be done bilaterally)
Downside is this can be negative due to “skip-lesions” being present.
Okay, we think they have it, how do I treat it?
According to NICE guidelines:
- GCA is a medical emergency and high dose steroids should be started (oral ASAP)
- If any visual changes – referral to an ophthalmologist for an urgent (same day) assessment
- Acute visual loss with GCA is treated with IV glucocorticoids usually
- If no visual symptoms – oral prednisolone daily 40-60mg
- Urgently discuss patient with Rheumatologist – ideally seen on the same day; in all cases must be seen within 3 days.
- Follow up will usually be with rheumatology however may exist in a shared care model as patients will likely need long-term steroid therapy (around 2 years).
Additional treatments in literature:
- Some studies showing azathioprine/cyclosporine but limited benefits recorded
- Usually will need steroids for a long duration, in order to try and wean patients off steroids can also use immunological therapy eg tocilizumab (IL-6 inhibitor)
- Vision loss can progress to the other eye
- Permanent vision loss
- Side effects of steroid therapy
Written by Dr Shuchi Kohli (FY2) & reviewed by Dr Kate Reed (ST4 Ophthalmology)
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