Table of contents
Giant cell arteritis
What's new
Updated 2024 EULAR guidelines for diagnostic imaging in giant cell arteritis.
Background
Overview
Definition
GCA is an inflammatory vasculitis affecting medium and large-sized arteries, with a predilection for the temporal arteries.
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Pathophysiology
In patients with GCA, the interaction of genetic and epigenetic factors causes pathologic activation of vascular dendritic cells, CD4 T cells, and macrophages, leading to granulomatous inflammation of the adventitia and hyperplasia of the intima. These vascular changes cause ischemic complications in tissues fed by medium and large-sized vessels.
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Epidemiology
In the US, the incidence of GCA is estimated at 18.9 cases per 100,000 person-years, while the prevalence is estimated at 228 persons per 100,000 population.
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Disease course
Ophthalmoplegia, vertebrobasilar insufficiency, central vision loss, stroke, and aortic aneurysm and rupture may occur as direct complications of vascular involvement.
2
Prognosis and risk of recurrence
Permanent vision loss occurs in approximately 15-20% of patients of GCA.
4
Guidelines
Key sources
The following summarized guidelines for the evaluation and management of giant cell arteritis are prepared by our editorial team based on guidelines from the European League Against Rheumatism (EULAR 2024,2020), the American Heart Association (AHA/ACC 2022), the American Heart Association (AHA/ASA 2021), the Vasculitis Foundation (VF/ACR 2021), the British Society for Rheumatology (BSR 2020), the Swedish Society of Rheumatology (SSR ...
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Screening and diagnosis
Diagnosis
As per BHPR/BSR 2010 guidelines:
Suspect large-vessel GCA in patients with prominent systemic symptoms, limb claudication or persistently high inflammatory markers despite adequate corticosteroid therapy.
B
Assess for the predictive features of ischemic neuro-ophthalmic complications of GCA.
B
ACR/EULAR classification criteria for giant cell arteritis
Entry criteria
Evidence of medium- or large-vessel vasculitis on imaging
Alternative diagnoses mimicking vasculitis are excluded
Age ≥ 50 years at the time of diagnosis
Clinical criteria
Morning stiffness in shoulders/neck
Sudden visual loss
Jaw or tongue claudication
New temporal headache
Scalp tenderness
Abnormal examination of the temporal artery (absent or diminished pulse, tenderness, or hard "cord-like" appearance)
Laboratory, imaging, and biopsy criteria
Maximum ESR ≥ 50 mm/hour or maximum CRP ≥ 10 mg/L before initiation of treatment
Positive temporal artery biopsy or halo sign on temporal artery ultrasound
Bilateral axillary involvement (luminal damage, such as stenosis, occlusion, or aneurysm)
FDG-PET activity throughout descending thoracic and abdominal aorta (FDG uptake in the arterial wall greater than liver uptake by visual inspection)
Diagnostic criteria are not met
Diagnostic investigations
Diagnostic imaging: as per EULAR 2024 guidelines, consider obtaining ultrasound of temporal and axillary arteries as the first imaging to assess mural inflammatory changes in patients with suspected GCA.
B
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Initial investigations
Assessment of comorbidities
Diagnostic procedures
Medical management
General principles: as per EULAR 2020 guidelines, manage patients with large vessel vasculitis based on a shared decision between the patient and the rheumatologist, considering efficacy, safety and costs.
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Setting of care
Corticosteroids
Tocilizumab
Methotrexate
Antithrombotics
Lipid-lowering agents
Surgical interventions
Specific circumstances
Patient education
Follow-up and surveillance
Assessment of treatment response: as per ACC/AHA 2022 guidelines, assess treatment efficacy periodically by monitoring clinical symptoms, inflammatory serum markers (CRP and ESR), and imaging (CT, MRI, or 18F-FDG-PET) in patients with active GCA.
B
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Follow-up
Management of relapse