Giant Cell Arteritis

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 symptomsSystemic symptoms
Headache Jaw claudication Pain in tongue Painful scalp/over temporal area Persistent pain Hardening of temporal artery Pulsating arteryCan 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.

Bloods: 

  • ESR (although not always elevated)
  • CRP
  • FBC

Imaging: 

  • 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)

Complications

  • Vision loss can progress to the other eye
  • Permanent vision loss
  • Stroke
  • Side effects of steroid therapy 

References

https://eyewiki.aao.org/Giant_Cell_Arteritis

https://next.amboss.com/us/article/1T02p2?q=giant%20cell%20arteritis#Z2d454d961f764521463153db6e93633c

https://cks.nice.org.uk/topics/giant-cell-arteritis/diagnosis/suspecting-giant-cell-arteritis/

Written by Dr Shuchi Kohli (FY2) & reviewed by Dr Kate Reed (ST4 Ophthalmology)

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