Giant Cell Arteritis

Overview

Giant cell arteritis (GCA) is a medical emergency. Early treatment with effective doses of glucocorticoids may prevent serious complications such as vision loss.

Symptoms/Presentation

• Headache - Although headache is present in two thirds of patients with GCA, it may be transient and is not always localised to the temporal regions.
• Constitutional symptoms - Over 90% of patients with GCA will describe constitutional symptoms, in particular fatigue. Malignancy must be excluded if marked weight loss is present or when concerning symptoms are detailed on systemic enquiry or examination.
• Visual symptoms - Patients may present with visual symptoms alone with no systemic symptoms.
• Jaw pain/ claudication - is the most specific symptom of GCA and may be associated with an increased risk of neuro-ophthalmic complications.

Complications

Complications of GCA include permanent visual loss, stroke and tissue necrosis. Treatment usually leads to rapid resolution of systemic symptoms. On the other hand, the use of steroids in uncertain cases before confirmation of GCA diagnosis may delay diagnosis and risk prolonged, unnecessary steroid use.

Patients with suspected GCA should be seen in the specialist clinic as soon as possible.

Please see the full referral pathways for NBT, BRI and WGH in the referral section below.

Characteristics suggestive of GCA

• Age over 50 years (typically over 60 years)
• Jaw/tongue claudication
• New headache
• Scalp pain and tenderness
• Temporal artery tenderness or reduced temporal artery pulse
• Limb claudication (associated large vessel vasculitis)
• Associated PMR symptoms and systemic upset
• Visual symptoms and signs – if present, contact the Eye Hospital. 
  • Diplopia - recent onset, transient or persistent
  • Transient loss of vision - severe sight loss in one eye with recovery within seconds or minutes
  • Loss of vision – recent onset, rapidly progressive sight loss over hours to days or sudden loss of vision in one or both eyes

Key differentials to consider

• Other causes of headaches e.g. cluster headaches, migraines - see the Headache page on Remedy for advice on diagnosis and management of other types of headache.
• Trigeminal neuralgia
• Herpes zoster/ shingles
• Acute angle closure glaucoma
• Temporomandibular joint pain, sinus disease, ear, dental problems
• Cervical spondylosis
• Transient ischaemic attacks (visual symptoms)
• Malignancies

Managing Giant Cell Arteritis

**See also the Referral Pathways in the section below for management as well as referral advice**

Clinical Assessment

1. Full clinical history including detailed systemic enquiry.
2. Cardiovascular examination to include palpation of peripheral pulses, auscultation of subclavian, axillary and brachial bruits and bilateral blood pressure measurement.
3. Temporal artery palpation (tenderness, thickening, beading, reduced or absent pulsation).
4. Cranial nerve examination (including ophthalmoscopy if available) but please do not delay referral of all patients with visual symptoms (diplopia or loss of vision) to ophthalmology for full assessment.
5. Full examination to exclude mimics (infection, malignancy or cervical spine pathology).

Blood tests

Please send CRP, ESR or PV, FBC, U&Es, LFTs, bone profile. (NB. If using UHBW lab then do CRP and ESR, and if NBT lab do CRP and PV)

Blood should be taken before or immediately after commencing high-dose glucocorticoids, unless there is evidence of critical ischaemia such as visual loss or diplopia and no immediate access to phlebotomy. If GCA is strongly suspected, the first dose of glucocorticoid can be given without waiting for laboratory results.

Please note, there can be a lag in the reporting of ESR of up to 3 days. Therefore, it is usually not necessary for these results to be chased in the out of hours period (but please organise follow up with the patient’s own GP for the next working day).

Inflammatory markers and starting steroids in GCA

Local clinicians advise that there are no cut off values for inflammatory markers in GCA and there are rare occasions where they can be normal. Initial diagnosis and treatment therefore depend on the pre-test probability of it being GCA as well as results of tests. If a clinician assesses the patient as high probability of GCA, then steroids should be started without delay. If a clinician feels there is a low probability, they may await further tests and/or refer before staring steroids. In these patients you would need to safety-net and advise that the patient should attend A&E if symptoms worsen. The on-call team can give advice regarding a patient if there is still uncertainty about starting steroids prior to their clinic review.

Referral pathways

Referral pathway for NBT

Referral Pathway for BRI

Referral Pathway for WGH

Once a patient is on steroids and has been reviewed by the secondary care team then they will advise about dose reductions regimes as guided by BSR guidelines.

Safety net advice

Advise the patient to seek (same day) medical attention (via Bristol Eye Hospital Emergency Care, or Bristol Royal Infirmary Emergency Department if BEH Emergency Care is closed) if they develop visual symptoms (visual loss, double vision).

If patients attend out of hours, advise the patient they will need to review with their usual GP the next working day to follow up on blood results and make referral to rheumatology.

Further advice

If you require further advice (and on-call rheumatology are not available), please contact the IUC Professional Line or on-call Medical SpR.

Resources

Clinical Knowledge Summary guidelines: CKS Guidelines for the Initial and Ongoing Management of Giant Cell Arteritis (web page)

BNSSG GCA Management and Referral Pathway above, Version 1.1 (May 2022) Authors: Dr Fang En Sin (NBT), Hannah Chapman (BNSSG), Dr Sarah Villar (NBT), Dr Harsha Gunawardena (NBT), Dr Joanna Robson (UHBW), Dr Stuart Webber (UHBW), Dr Elizabeth Perry (UHBW).

Reference: British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis January 2020 (https://academic.oup.com/rheumatology/article/59/3/e1/5714024#246013768)

Efforts are made to ensure the accuracy and agreement of these guidelines, including any content uploaded, referred to or linked to from the system. However, BNSSG ICB cannot guarantee this. This guidance does not override the individual responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or guardian or carer, in accordance with the mental capacity act, and informed by the summary of product characteristics of any drugs they are considering. Practitioners are required to perform their duties in accordance with the law and their regulators and nothing in this guidance should be interpreted in a way that would be inconsistent with compliance with those duties.

Information provided through Remedy is continually updated so please be aware any printed copies may quickly become out of date.