• GCA is the most common large vessel vasculitis

• It is a medical emergency and can cause blindness in up to 20% of patients

• Patients with suspected GCA should be commenced immediately on steroids and be referred, via a fast-track pathway, to Rheumatology for further investigation and management.

• Patients with visual disturbance require a same day referral to ophthalmology, in addition to Rheumatology.

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Abbreviations

Giant cell arteritis (GCA), also known as Temporal Arteritis, is the commonest large vessel vasculitis with a predilection for temporal arteries and the aorta. GCA is a medical emergency. It is a common cause of acute blindness with visual loss occurring in up to 20% of patients with GCA. If one eye is affected there is a high risk (up to 50%) of bilateral visual loss and total blindness without appropriate treatment. GCA can also be associated with aortitis which can present as systemic upset, limb claudication, bruits and asymmetrical pulses and blood pressure.

These may not all be present. GCA may not present in a textbook way.

Suspicion of GCA

• Age over 50? (GCA extremely unlikely under the age of 50)
• Abrupt onset unilateral/temporal headache
• Scalp tenderness
• Jaw / tongue claudication
• Visual disturbance (amaurosis fugax / diplopia / blurred vision)
• Symptoms of PMR
• Limb claudication

If the patient does not meet the criteria above, consider an alternative diagnosis.

Consider wide range of differential diagnoses if not classical features, particularly if CRP normal or age below age 50. For example:

• Herpes Zoster (Shingles)
• cluster headache or migraine
• acute angle closure glaucoma
• retinal transient ischaemic attack and embolic visual deficits
• TMJ pain
• sinus disease
• ear problems
• cervical spondylosis
• upper cervical spine disease
• myeloma
• serious intracranial pathology
• connective tissue disease

(NICE CKS GCA).

Urgent investigations

FBC, U&E, LFT, bone profile, CRP, ESR, immunoglobulins, HbA1C

Urgent same day referral to ophthalmology

in addition to initial investigations - Wexham/Frimley Eye casualty, ext. 136854

Initiate treatment

Do not delay starting steroids in those with strong clinical suspicion for GCA or in context of visual disturbance

• Prednisolone 40-60mg daily

  • Absence of cranial ischaemic symptoms, e.g., visual disturbance and jaw claudication – suggest 40mg

  • Presence of cranial ischaemic symptoms – 60mg (acute visual disturbance must be d/w Ophthalmology as may warrant iv methylprednisolone)

  • Co-prescription of PPI, bone protection (calcium/vitamin D and bisphosphonate), provided no contraindication.

Refer to Rheumatology

History

• Enquire about comorbidities and medications that might predispose to glucocorticoid-related adverse effects, including infection, hypertension, diabetes, osteoporosis, low-trauma fracture, dyslipidaemia, peptic ulcer, and psychiatric adverse effects

• Ask about features that may suggest an alternative diagnosis, e.g., neurological deficits, very severe constitutional symptoms or localised ear, nose, and throat signs

Investigations

• Consider screening tests for serious infection prior to initiating high dose steroid (e.g., urine dipstick, CXR)

• Further investigations (Rheumatology will arrange)

  • Temporal artery ultrasound (should be done within one week of steroid initiation)

  • Temporal artery biopsy (requested by Rheumatology via the General surgeons)

• If suspicion of GCA is high in context of normal inflammatory markers, please ask for an urgent rheumatology opinion

Initial treatment

• Do not delay starting steroids in those with strong clinical suspicion for GCA, or in context of visual disturbance and suspected GCA

• Recommended starting dose of steroids:

  • Absence of cranial ischaemic symptoms (jaw / tongue claudication or acute visual disturbance): 40mg prednisolone daily

  • Presence of cranial ischaemic symptoms: 60mg prednisolone daily

• The presence of acute visual disturbance will need immediate discussion with Ophthalmology (may warrant intravenous methylprednisolone treatment)

• All patients with need co-prescription of a PPI, bone protection (calcium/vitamin D and bisphosphonate) provided there are no contra-indications.

A typical glucocorticoid tapering schedule for GCA

• Aim to reach 20 mg prednisolone once the patient has been in remission for 4–8 weeks
• If symptoms suggestive of GCA relapse occur during taper step up as below

Examples of symptoms that may signify relapse of GCA during glucocorticoid taper that require further evaluation and, if judged to be due to GCA relapse, escalation of glucocorticoid treatment

2010 BSR Guideline for GCA

Methotrexate

MTX might be considered for GCA, in combination with a glucocorticoid taper, in patients at high risk of glucocorticoid toxicity or who relapse. There is insufficient evidence to recommend any other oral immunosuppressive agent in GCA, including azathioprine, leflunomide or mycophenolate mofetil. (BSR Guideline 2020)

Tocilizumab

Tocilizumab can be considered for GCA, in combination with a glucocorticoid taper, especially in patients at high risk of glucocorticoid toxicity or who relapse. TNF inhibitors are not recommended in GCA. (BSR Guideline 2020)

Tocilizumab, when used with a tapering course of glucocorticoids (and when used alone after glucocorticoids), is recommended as an option for treating giant cell arteritis in adults, only if:

• they have relapsing or refractory disease

• they have not already had tocilizumab

• tocilizumab is stopped after 1 year of uninterrupted treatment at most and

• the company provides it with the discount agreed in the patient access scheme.