Giant cell arteritis/Temporal Arteritis

Background/Epidemiology

  • GCA is a chronic vaculitis of medium-large sized arteries and arch of the aorta.
  • One of the most commoon causes of acute blindness (permanent/irreversible)
    • visual loss occurs in up to 20% of patients with GCA (delayed diagnosis and treatment
    • MEDICAL EMERGENCY- requires urgent referral to ophthalmology or rheumatology

Presentation

  • Acute onset, unilateral (often temporal) headache
    • Scalp pain (may cause problems combing hair
  • Jaw and tongue claudication (difficulty chewing)
  • Symptoms of polymyalgia rheumatica e.g. pain, stiffness, tenderness in procximal muscle groups (50%)
  • Constitutional symptoms e.g. fever, weight loss, tiredness
  • Visual symptoms
    • Classically ‘curtain’ like loss of visual field (amaurosis fugax), although blurring/double vision also possible
  • Other symptoms of claudication (large vessel involvement) e.g. paraesthesia or pain of the hands/feet/calves on exertion; asymmetrical pulses; bruits

Investigations

  • A raised ESR/PV is highly sensitive for GCA (95% will have an ESR >50mm/hour)
  • Temporal artery biopsy should be performed in patients with clinically suspected GCA within one week
    • NB biopsy is NOT required if patients respond to empirical steroid treatment
  • Imaging (only if there are symptoms/signs of large vessel involvement or severe systemic symptoms; or persistently high ESR/PV despite treatment)
    • CT (FDG-PET) or duplex USS (or MRI)

Diagnostic criteria

  • Any 3 of
    • Age >50
    • New headache
    • Abnormal temporal artery (e.g. tender, rubbery)
    • Raised ESR (>50) or PV
    • Positive temporal artery biopsy

Management

  • Steroids should be started as soon as the diagnosis is suspected
    • Start high-dose (60-80mg) for patients with claudication/visual symptoms/critical ischaemia
    • Medium dose steroids can be used for uncomplicated GCA (40mg)
    • Dose can be reduced after 4 weeks if both symptoms and serum inflammatory markers have normalised
      • Reduce by 10mg every 2 weeks to 20mg/day; then by 2.5mg every 2-4 weeks to 10mg/day; then 1mg every 1-2 months- provided there is no relapse
      • Assess patients at week 1, 3, 6 then every 3 months for first year (test FBC, U&Es, ESR/PRV, CRP, glucose)
      • NB 30-50% of patients can stop taking steroids after 2 years, but many require longer.  Patients should be educated about the long-term use of steroids and risk of adrenal suppression as much as other side-effects
        • If patients do relapse with complications, increase to start dose i.e. 60mg.  If without complications, revert to maintenance dose i.e. lowest dose where symptoms were controlled.
  • Also start low dose aspirin, bone protection (bisphosphonates; calcium and vit D supplementation) and a PPI (unless there are contraindications
  • Offer patients vaccinations for influenza and pneumococcal vaccination
  • Also offer a CXR (or abdo CT) every 2 years for signs of AAA/aortic dissection
Advertisements

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out /  Change )

Google+ photo

You are commenting using your Google+ account. Log Out /  Change )

Twitter picture

You are commenting using your Twitter account. Log Out /  Change )

Facebook photo

You are commenting using your Facebook account. Log Out /  Change )

w

Connecting to %s