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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.
2
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.
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Prognosis and risk of recurrence
Permanent vision loss occurs in approximately 15-20% of patients of GCA.
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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 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 European League Against Rheumatism (EULAR 2020,2018), the Swedish Society of Rheumatology (SSR ...
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Screening and diagnosis

Diagnosis
As per EULAR 2018 guidelines:
Consider establishing the diagnosis of GCA without obtaining additional tests (biopsy or further imaging) in patients with high clinical suspicion and a positive imaging.
C
Recognize that the diagnosis of GCA is unlikely in patients with a low clinical probability and a negative imaging. Make additional efforts toward a diagnosis in all other cases.
B
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Diagnostic investigations

Diagnostic imaging: as per ACC/AHA 2022 guidelines, obtain prompt evaluation of the entire aorta and branch vessels with MRI or CT, with or without 18F-FDG-PET, in patients with large vessel vasculitis.
B

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  • Initial investigations

  • Assessment of comorbidities

Diagnostic procedures

Temporal artery biopsy: as per ACR/VF 2021 guidelines, consider performing an initial unilateral rather than bilateral temporal artery biopsy in patients with suspected GCA.
C
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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

Reconstructive surgery: as per ACC/AHA 2022 guidelines, consider performing elective endovascular or open surgical intervention to treat aortic and branch vessel complications in patients with GCA in remission.
C

Specific circumstances

Patients with stroke or TIA: as per AHA/ASA 2021 guidelines, initiate immediate oral high-dose corticosteroids to reduce recurrent stroke risk in patients with ischemic stroke or TIA and symptoms attributed to GCA.
B
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Patient education

Patient education: as per EULAR 2020 guidelines, ensure that patients have access to education focusing on the impact of large vessel vasculitis, its key warning symptoms and treatment (including treatment-related complications).

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