Guidelines for assessment, investigation and acute management of Giant Cell Arteritis

Background

Giant Cell Arteritis (GCA) is a chronic vasculitis of large and medium sized blood vessels. The most immediate worry is visual loss with a proportion of patients having permanent deficit. Due to the heterogeneity of the condition patients may present in a variety of ways to a spectrum of clinicians including GPs, rheumatologists, acute physicians, elderly care physicians and ophthalmologists. Temporal artery biopsy is the ‘gold standard’diagnostic test and is extremely helpful in the management of patients and who do not respond to long term treatment as would be expected. The aims of these guidelines areto standardise the diagnostic pathway and management of these patients.

Clinical Features

  1. Age of onset > 50 yrs ( GCA almost never occurs below this age)
  2. Abrupt onset ofsevere headache (usually temporal).
  3. ESR > 50 ( or equivalent raise in plasma viscosity/CRP)
  4. Polymyalgia Rheumatica-like symptoms
  5. Temporal artery - look for:
  6. tenderness
  7. reduced or absent TA pulsation
  8. prominent or beading temporal artery
  9. Jaw or tongue claudication( i.e. abrupt onset of pain on chewing or talking) *
  10. Systemic illness (fever, anorexia, weight loss etc.)
  11. Visual disturbanceincluding diplopia*
  12. Upper cranial nerve palsies
  13. Limb claudication or other evidence of large vessel involvement

* strong predictors of neuro-opthalmic complications, requiring higher steroid doses (often parenteral steroids)

Important: the differential diagnosis is wide. Other diagnoses to consider include malignancy(especially in the elderly) or infection. Other forms of vasculitis may present with involvement of the cranial arteries.

Assessment:

  1. Patients with suspected GCA should be referred to Medicine to be seen at 9am by the on-call medical registrar (in order to facilitate referral for biopsy). Please refer to the medical registrar on-call if your patient has symptoms and signs of GCA as per the clinical features listed above.
  1. A full physical examination should be performed including palpation of all major pulses, auscultation for bruits and blood pressure measurement in both arms. Fill out GCA diagnostic pathway.
  1. Bloods should be requested for FBC, U&Es, LFTs, ESR (or plasma viscosity) and CRP.
  1. Check a urine dipstick.
  1. All patients should have a chest X-ray

Acute Management:

Temporal Artery Biopsy:

a)all patients with suspected GCA should be started on steroids (see below) and considered for a TA biopsy.

b)Patients should be referred to the medical registrar on-callfor assessment who will refer for a TA biopsy if required.

c)Use the yellow day-case surgery formto refer for TA biopsy (these can be found in ED or EAU and the electronic version is kept on the emergency drive).

d)The referral form needs to be taken to or sent to General Surgery Admissions, ENT reception, level 2 (office behind ENT reception) or you can telephone 01803-656316 or email .

e)Day-case surgery will contact the patient directly and will undertake to carry out the biopsy within 3-5 working days on the consultant general surgical list (usually Mr. Srinivas or Mr. Johnson’s lists).

f)A referral to Rheumatology also needs to be made and sent to the Rheumatology Department (use a white in-patient referral form or GCA referral form and state the date the patient has been referred for biopsy).

Ophthalmology review:refer all patients with ocular symptoms for urgent review (N.B. if visual loss the patient might need to be admitted for parenteral steroids).

Steroids:all patients should be started on Prednisolone 60mg daily for 6 weeks initially. Patients with ocular symptoms and/or symptoms of jaw claudication should be commenced onPrednisolone 80mg daily.

Bone Protection: all patients should be commenced on supplemental calcium and vitamin D +/-a bisphosphonate. Rheumatology will organise a DEXA scan and start the bisphosphonate as required.

Aspirin: should be considered in all patients with GCA as anti-platelet therapy for prevention of ischaemic complications e.g. CVA, TIA, (assuming no contraindications).

Gastric protection: treatment with a proton pump inhibitor or H2 antagonist should also be considered especially if Prednisolone and Aspirin are co-prescribed.

Rheumatology FU: refer all patients to Rheumatology for outpatient follow up. Day-case surgery will organise for the TA biopsy results to be sent directly to Rheumatology.Biopsy results will be reviewed and further management including steroid tapering will be undertaken.

Patient information: can be found on the ARC website at under Polymyalgia Rheumatica which can be printed off for patients.

If there are any doubts with regard to diagnosis/management please discuss with a Rheumatologist urgently (ext: 54832).

Special situations:

  1. Visual loss in the setting of new diagnosis of GCA – consider admission for parenteral steroids and urgent ophthalmology review.
  1. Limb claudication – please consider discussion with a vascular radiologist with regard to best modality for further imaging and urgent vascular surgical review.

K. Mackay & J. King –updated March 11