Recommendations
Key Recommendations
Consider GCA in a patient aged >50 years who presents with signs and symptoms such as new onset of headache, limb, jaw, or tongue claudication, and acute visual symptoms, along with unexplained raised inflammatory markers.[35][36]
GCA is a medical emergency and is associated with a risk of permanent sight loss and (more rarely) stroke.[35][36][37]
Take blood for full blood count, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) before starting treatment (as the inflammatory markers decrease with glucocorticoid use).[36]
CRP and ESR are typically raised and platelet count may be elevated in people with GCA. In practice, CRP may be a more useful and specific test than ESR.
If there is evidence of critical ischaemia, urgent assessment must be made with a view to commencing immediate treatment.
Do not wait for the test results before starting a high-dose glucocorticoid in patients you strongly suspect to have GCA.[36]
Confirm the diagnosis in all patients with suspected GCA with ultrasonography and/or a temporal artery biopsy.[35][36]
If available, use rapid-access vascular ultrasonography of the temporal and axillary artery first line to diagnose suspected GCA.[35][36][38]
In patients in whom there is a high clinical suspicion of GCA and a positive imaging result, the diagnosis of GCA may be made without an additional test (biopsy or further imaging).[38]
In patients with a low clinical probability and a negative imaging result, the diagnosis of GCA can be considered unlikely.[38]
Consider a temporal artery biopsy if:
Expertise for ultrasonography is not available
The patient’s pre-test probability is high (typical features of GCA with raised inflammatory markers) but the ultrasonography is normal.[36]
Refer the patient with suspected GCA to a specialist to confirm the diagnosis and make a longer term definitive plan.[35][36] This is usually a rheumatologist, but may be an ophthalmologist or neurologist as needed.
Fast-track GCA assessment pathways are increasingly common in the UK and elsewhere, and patients should be discussed as soon as possible with the specialist team. Patients should be evaluated by a specialist ideally on the same day.[37]
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 day by an ophthalmologist.[36][37][39][40]
Suspect GCA in a patient aged >50 years who presents with signs and symptoms such as new onset of headache, scalp tenderness, limb, jaw, or tongue claudication, and acute visual symptoms, along with unexplained raised inflammatory markers.[35][36] Headache is almost universally present in patients with GCA. Scalp tenderness is common but non-specific. Jaw or tongue claudication is specific but uncommon.[41] Constitutional symptoms, such as fever, fatigue, night sweats, and weight loss may also be present.[36]
Around one third of patients have pain and stiffness in the neck and proximal upper and lower extremities (which may be worse in the morning); this may also be a symptom for polymyalgia rheumatica.
Neurological manifestations can occur, though uncommonly, and may include stroke or transient ischaemic attack.[2][4]
Rarely, dental pain, swallowing difficulty, tongue pain, or infarction of the tongue may be present.[2][5] Dental practitioners may be the first port of call for patients with oral manifestations.
Extracranial GCA predominantly affects the aorta and proximal branches and is less common than cranial GCA. It can present as an unexplained systemic illness or fever of unknown origin with elevated levels of inflammatory markers without headache, jaw claudication, shoulder or hip girdle stiffness, or visual disturbances.
Patients with axillary or subclavian artery involvement (clinically apparent in approximately 10% to 15% of patients) may present with claudication of the upper extremities.[7]
Practical tip
Visual disturbances, limb, jaw, or tongue claudication, and constitutional symptoms, such as fever, fatigue, and weight loss, may go unrecognised as diagnostic factors for GCA if unaccompanied by headache.[42][43]
Investigations for GCA should form part of the protocol for patients with pyrexia of unknown origin.
Some patients who predominantly have shoulder girdle pain and stiffness may be mistakenly diagnosed as having polymyalgia rheumatica if investigations for GCA have not been performed.
GCA is a medical emergency and is associated with a risk of permanent sight loss and (more rarely) stroke.[35][37]
Refer the patient with suspected GCA to a specialist (usually a rheumatologist, but may be an ophthalmologist or neurologist, as needed) to confirm the diagnosis with vascular ultrasonography and/or temporal artery biopsy.[35][36] Use a ‘fast-track’ referral pathway for urgent specialist evaluation if available, but do not delay treatment.[36]
Patients should be evaluated by a specialist ideally on the same day, with a view to a definitive plan within 3 days.[36][37]
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 day by an ophthalmologist.[36][39][40]
Multidisciplinary input may be required to interpret investigation results or consider other diagnoses.[36]
Ask the patient about:[36]
Symptoms resulting from ischaemia of specific arteries
Superficial temporal artery - scalp tenderness
Maxillary artery - jaw claudication, toothache, sudden drop in hearing, severe headache
Posterior auricular artery - pain around the ear
Occipital artery - posterior scalp tenderness
Facial artery - swallowing difficulty
Lingual artery - painful tongue, tongue claudication
Superior thyroid artery - hoarseness of voice
Carotid artery - neck pain and tenderness
Vertebral artery - neck pain, unsteadiness of gait
Limb claudication and constitutional symptoms that may point to extracranial arterial involvement: weight loss, loss of appetite, drenching night sweats
Visual involvement: double vision, loss of vision in any or all parts of the visual field
Visual symptoms may be transient[43]
History suggesting alternative diagnosis: bleeding or crusting from nose, change in bowel/bladder habit, swellings or lumps in neck, productive cough, burning or stinging of urine, rashes
History of comorbidities that may be of importance for medication: diabetes mellitus, hypertension, heart failure, osteoporosis, glaucoma, cataracts, acid-peptic disease, hiatus hernia.
On physical examination, look for abnormalities of the temporal, occipital, and facial arteries including thickening, tenderness, and nodularity.[36] Fundoscopic abnormalities may include pallor and oedema of the optic disc, possibly with cotton-wool patches and haemorrhages.
Check for signs and symptoms indicating involvement of extracranial arteries, such as bruits over the subclavian and axillary arteries.
Check the pulses over the common carotid and radial arteries; they may be asymmetrical or completely absent
Check blood pressure across both arms.[36]
Patients with significant stenoses (approximately 10% to 15% of patients) may have asymmetric blood pressures or decreased pulses.[7]
Check the patient’s height and weight.[36]
Consider referral for an ophthalmological examination if there are any ophthalmic symptoms.
Take blood for full blood count, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) before starting high-dose glucocorticoids (see Management recommendations).[36][39] CRP and ESR are typically raised and platelet count may be elevated in people with GCA. However, inflammatory markers decrease once glucocorticoid therapy is started.[35][36] In practice, CRP may be a more useful and specific test than ESR.[1]
Do not wait for the test results before starting a high-dose glucocorticoid in patients you strongly suspect to have GCA.[36]
Consider further tests to establish major organ system function, such as renal and liver function tests, plasma glucose, calcium, and alkaline phosphatase.[36] Mild abnormalities are common and should resolve with treatment. Persisting or severe abnormalities should prompt a screen for an underlying cause, including malignancy and hepatitis, symptoms of which may mimic GCA in some patients.
Practical tip
Establish a baseline for the patient’s glycaemic status prior to starting long-term glucocorticoid therapy, which is a risk factor for developing type 2 diabetes.
Confirm the diagnosis in all patients with suspected GCA, either with imaging such as ultrasonography or [18F]-fluorodeoxyglucose (FDG)-PET , or with a temporal artery biopsy, depending on local availability.[35][36]
Ultrasonography
If available, use rapid-access vascular ultrasonography of the temporal and axillary artery first line to diagnose suspected GCA.[35][36][38]
Ultrasonography reduces the number of patients who need a temporal artery biopsy and has the advantage of being non-invasive and more cost effective, and the rapid bedside availability may help to reduce sight loss.[36][44][45][46][47]
Ultrasonography is best performed in validated centres.[36][48]
In patients with GCA, ultrasonography may show wall thickening (halo sign), stenosis, or occlusion. A non-compressible halo sign is the finding that most indicates GCA.[38] Diagnostic ultrasonography for GCA should be performed within 7 days of starting glucocorticoids.[37] Rapid access is important as the ultrasound ‘halo’ diminishes in size once glucocorticoid therapy has been started.[44] Ultrasonography appearances may normalise 2-4 weeks after commencing glucocorticoids.[37][49]
In patients in whom there is a high clinical suspicion of GCA and a positive imaging result, the diagnosis of GCA may be made without an additional test (biopsy or further imaging).[38] In patients with a low clinical probability and a negative imaging result, the diagnosis of GCA can be considered unlikely. In all other situations (including the case of an inconclusive imaging result), further investigations to reach a diagnosis are required.[38]
Temporal artery biopsy
Consider a temporal artery biopsy if:
Expertise for ultrasonography is not available
The patient’s pre-test probability is high (typical features of GCA with raised inflammatory markers) but the ultrasonography is normal.[36]
A biopsy should be performed by a surgeon experienced in this procedure.[36] A positive temporal artery biopsy showing features of intramural inflammation characteristic of GCA, such as giant cells or panarteritis, confirms the diagnosis of GCA.[27][36][50] Isolated vasa vasorum vasculitis is not diagnostic of GCA.[36]
The biopsy is performed on the side with abnormal clinical findings (if present). Specimens should be no shorter than 1 cm (post-fixation) due to the possibility of skip lesions.[35][36] In one study, the diagnostic yield was improved by having a specimen of at least 5 mm length, which was examined at multiple levels.[51]
Do not delay treatment while waiting for the biopsy to be performed (see Management recommendations).[36] The biopsy should be harvested within 4 weeks of starting glucocorticoids.[37] However, even after several weeks of glucocorticoid therapy, temporal artery biopsy may still yield a diagnosis of arteritis.[52][53] Indeed, the majority of temporal artery biopsies (9 out of 12) performed in a small cohort of patients with treated GCA had persistent histopathological evidence of vasculitis even after 6 months of glucocorticoid therapy.[54] Ongoing vasculitis was apparent in 4 out of 9 biopsies after 12 months of treatment.
Temporal artery biopsy is less helpful in patients with extracranial GCA and may be negative in up to 50% of these patients.[55] This may be due to skip lesions, involvement of arteries besides the superficial temporal artery, duration of treatment with prednisolone prior to procedure, incorrect operative technique, or inadequate examination of the biopsy specimen. However, even people thought to have extracranial disease may have a positive temporal artery biopsy in some cases and thorough investigation is always warranted.
Other imaging
High-resolution magnetic resonance imaging (MRI) or FDG-PET can be used as alternatives to ultrasound for the assessment of cranial arteries in patients with suspected GCA.[38] Assess suspected extracranial GCA using alternative imaging modalities such as [18F]-FDG-PET, or alternatively magnetic resonance angiography (MRA) or computed tomography angiography (CTA).[36][38] Sensitivity of [18F]-FDG-PET is affected by corticosteroids so should ideally be performed before initiating treatment.
[18F]-FDG PET-CT scanning is of great value to assess for extracranial vasculitis.[38] The diagnostic yield falls rapidly after the onset of glucocorticoid therapy and it should be done within 7 days of the request to avoid false negative results.[37]
It is of value in defining the extent of disease and in the demonstration of malignancies, which form an important differential diagnosis of GCA.
3 Tesla MRI of the cranial arteries is not widely available, but can be used as a diagnostic test or if neither vascular ultrasonography or temporal artery biopsy are available.[36][38] It would not be a first choice due to the risk of false-positive results, cost, availability, and the expertise required, and also the possibility of adverse effects to the contrast agents used.[36]
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