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Overview

This summary outlines the general principles of identifying and treating patients with giant cell arteritis in primary care and specialist settings. It is recommended that general practitioners refer patients with suspected giant cell arteritis to a clinician with appropriate specialist expertise. For the full set of recommendations, please refer to the full guideline.

View this summary online at guidelines.co.uk/455340.

Background

Giant cell arteritis (GCA) is a large vessel vasculitis affecting older people, with the highest incidence among persons 70–79 years of age.

In GCA there is inflammation within the walls of medium- and large-sized arteries, with associated intimal hyperplasia. The ischaemia to end organs results in characteristic clinical features such as jaw or limb claudication. Visual loss or stroke may occur in GCA, attributed to vascular occlusion; most GCA-associated visual loss occurs prior to glucocorticoid treatment or shortly after treatment initiation, underlining the importance of immediate treatment if the disease is strongly suspected.

Diagnosis

How quickly should patients with suspected GCA be referred for evaluation?

GCA is a medical emergency. Each local healthcare organisation should have information available to frontline clinicians, such as general practitioners and clinicians working in acute care, on how to refer patients with suspected GCA urgently for local specialist evaluation. Patients should be evaluated by a specialist ideally on the same working day if possible and in all cases within three working days.

To whom should patients with suspected GCA be referred?

Patients with suspected GCA should be evaluated by a clinician with appropriate specialist expertise, usually a rheumatologist. Patients presenting with a history of new visual loss (transient or permanent) or double vision should be evaluated as soon as possible on the same calendar day by an ophthalmologist.

How should GCA be diagnosed?

Strong recommendation: patients with suspected GCA should have a confirmatory diagnostic test. This could be either a temporal artery biopsy at least 1 cm in length or an ultrasound of the temporal and axillary arteries, or both. 

Conditional recommendation: 18F-FDG-PET, MRA, CTA or axillary artery ultrasound may be used to evaluate involvement of the aorta and its proximal branches.

Table 1: A proposed list of clinical assessments that could be carried out at or near diagnosis of GCA

History and examinationInvestigations
  • Height and weight

  • Features of GCA relevant to prognosis: fever, sweats or weight loss; ischaemic manifestations (jaw claudication, tongue claudication)

  • Signs and symptoms indicating involvement of extracranial arteries, e.g. bruits, different blood pressures in the two arms, and limb claudication

  • Ophthalmological evaluation for patients with transient or permanent visual loss or diplopia

  • History of 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

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

  • Measures of activity of GCA: laboratory markers of inflammation (CRP for all patients, plus either ESR or plasma viscosity) and full blood count (platelet count may be elevated in GCA)

  • Consider serum protein electrophoresis and urine Bence–Jones protein/serum free light chains if ESR elevated out of proportion to CRP

  • Baseline laboratory tests of major organ system function (plasma glucose, renal and liver function tests, calcium and alkaline phosphatase)

  • Screening tests for risk of serious infection[A] (may include urine dipstick, chest radiograph and tests for latent tuberculosis according to local or national protocol)

  • Screening tests for osteoporosis risk[A] (may include TSH, vitamin D, bone density test, DXA)

[A] Screening tests for infection and osteoporosis to be considered in light of relevant local and national guidelines.

Abbreviations: TSH=thyroid stimulating hormone; DXA=dual-energy X-ray absorptiometry.

Table reproduced with kind permission from Mackie, Dejaco, Appenzeller et al and the British Society for Rheumatology.

Treatment

How should suspected GCA be treated?

Patients in whom GCA is strongly suspected should be immediately treated with high-dose glucocorticoids.

What evaluations should be performed when starting treatment?

When starting glucocorticoids for suspected GCA, diagnostically relevant symptoms and signs should be documented. Blood should be taken for full blood count, CRP and ESR before or immediately after commencing high dose glucocorticoids. If GCA is strongly suspected, the first dose of glucocorticoid can be given without waiting for laboratory results.

What evaluations should be performed soon after starting treatment?

Patients treated for GCA should be evaluated for features of the disease relevant to the prognosis, such as clinical and laboratory features of a marked inflammatory response at diagnosis, ischaemic manifestations such as transient visual loss or jaw/tongue claudication, and signs or symptoms indicating involvement of the aorta and its proximal branches, and for comorbidities relevant to treatment, such as diabetes mellitus, hypertension, and bone fracture risk.

What is the best dose and route of initial glucocorticoid therapy for GCA in the absence of ischaemic visual manifestations?

The standard initial glucocorticoid dose for GCA is 40–60 mg oral prednis(ol)one per day. 

What is the best dose and route of initial glucocorticoid therapy for GCA in the presence of ischaemic visual manifestations?

GCA patients with acute or intermittent visual loss may initially be given 500 mg–1 g intravenous methylprednisolone daily for up to three consecutive days before commencing oral prednis(ol)one therapy. If intravenous therapy is not immediately possible, this should not delay initiation of oral prednis(ol)one.

How should ongoing management of GCA be individualised?

Full assessment of the disease and comorbidities and consideration of the patient’s personal priorities should inform decisions about glucocorticoid tapering and initiation of additional treatments such as glucocorticoid-sparing therapies. Involvement of and clear communication with primary care physicians is critical, especially for management of multimorbidity.

How should glucocorticoid dose be tapered in GCA?

Glucocorticoid dose should be tapered to zero over 12–18 months, providing there is no return of GCA symptoms, signs or laboratory markers of inflammation. A more rapid dose reduction is appropriate for patients at high risk of glucocorticoid toxicity and/or those receiving concomitant glucocorticoid-sparing therapy.

Table 2: A typical glucocorticoid tapering schedule for GCA

Daily prednisolone doseExample rate of reduction in daily prednisolone doseNotes

40–60 mg oral prednisolone: initial dose for patients with active GCA

Continue at same dose until GCA symptoms and acute phase markers resolve

Purpose: induction of clinical remission

In clinical remission and >20 mg prednisolone

Reduce daily dose by 10 mg every two weeks

Aim to reach 20 mg prednisolone once the patient has been in remission for four to eight weeks

In clinical remission, >10 mg prednisolone but <20 mg

Reduce daily dose by 2.5 mg every two to four weeks

If symptoms suggestive of GCA relapse occur during taper, consult Table 3

In clinical remission and on ≤10 mg prednisolone

Reduce daily dose by 1 mg every one to two months

Table reproduced with kind permission from Mackie, Dejaco, Appenzeller et al and the British Society for Rheumatology.

Table 2 is an example of a typical glucocorticoid taper schedule, based on that described in the 2010 BSR guidelines for GCA and similar to the control arm of a recent GCA clinical trial. High-quality evidence comparing different glucocorticoid taper schedules in GCA is not available. Alternative approaches include, for example, reducing prednisolone by 10 mg/week in patients who are in remission at >20 mg daily and/or reducing the dose slower than stated here in patients who are on ≤5mg daily. In all cases, taper schedules should be individualised based on the patient. For relapse management, see Table 3.

What dosing frequency of oral glucocorticoid should be used in GCA?

Patients should be prescribed a single daily dose of glucocorticoid rather than alternate day dosing or divided daily dosing.

When should further, non-biologic immunosuppression be added to glucocorticoid therapy for GCA?

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.

Which biologic agents can be used for GCA in addition to standard therapy?

Strong recommendation: 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.

Should anticoagulant or antiplatelet agents be given for GCA?

The routine use of antiplatelet or anticoagulant agents for GCA is not recommended. 

Should cholesterol-lowering agents be given for GCA?

The routine use of cholesterol-lowering agents such as statins for GCA is not recommended.

What plans should be made for possible future GCA relapses?

During glucocorticoid taper and after glucocorticoid cessation, patients should be informed what symptoms may suggest GCA relapse and what action the patient should take in these circumstances, including the first point of contact for medical advice and how to contact the team providing specialist care.

Table 3: 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

SymptomPossible significance in a patient with GCAAction to consider if symptom is judged to be due to GCA relapse

Return of headache symptoms

Possible GCA relapse without ischaemic manifestations

Return to previous higher prednisolone dose

Jaw or tongue claudication

Possible GCA relapse with ischaemic manifestations

Consider high-dose oral prednisolone (40–60 mg) with or without glucocorticoid-sparing agent

Weight loss, fever, night sweats, anaemia, persistent acute phase response, new/recurrent PMR symptoms, limb claudication, abdominal pain, or back pain

Possible GCA-related inflammation of the aorta and/or its proximal branches

Investigate with vascular imaging (MRI, CT or FDG-PET/CT); consider increasing oral prednisolone and/or adding glucocorticoid-sparing agent

Table reproduced with kind permission from Mackie, Dejaco, Appenzeller et al and the British Society for Rheumatology.

Table 3 outlines how new symptoms in GCA patients, in the absence of other risk factors or significant comorbidities, may influence management decisions. New visual loss or diplopia should be urgently evaluated by an ophthalmologist. Acute phase markers should be measured and, if found to be elevated, may increase the clinical suspicion of GCA relapse. At present, the only agents with any evidence for glucocorticoid-sparing in GCA are methotrexate and tocilizumab.

What education should patients be offered?

All patients with GCA should be provided with information about GCA and its treatment. Patients should receive advice on diet, physical activity and stopping smoking.

 

Full guideline:

academic.oup.com/rheumatology/article/59/3/e1/5714024.

Mackie SLDejaco C,Appenzeller S et al.  British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology 2020: 59(3); e1–e23

Published date: 23 January 2020.