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Giant cell arteritis

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.
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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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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 Vasculitis Foundation (VF/ACR 2021), the American Heart Association (AHA/ASA 2021), the British Society for Rheumatology (BSR 2020), the European League Against Rheumatism (EULAR 2020; 2018), the Swedish Society of Rheumatology (SSR 2019), the French Study Group for Large Vessel Vasculitis (GEFA 2016), the Society for Cardiovascular Angiography and Interventions (SCAI/STS/SVM/AATS/SCA/AHA/ACR/ACC/ASA/SIR 2010), and the British Society for Rheumatology (BSR/BHPR 2010).
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Guidelines

1.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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2.Diagnostic investigations

Diagnostic imaging: as per ACC 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

3.Diagnostic procedures

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

General principles: 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

5.Surgical interventions

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

6.Specific circumstances

Patients with stroke or TIA: 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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7.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).

8.Follow-up and surveillance

Assessment of treatment response: as per ACC 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