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2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for giant cell arteritis

2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for giant cell arteritis
Considerations when applying these criteria
  • These classification criteria should be applied to classify the patient as having giant cell arteritis when a diagnosis of medium-vessel or large-vessel vasculitis has been made
  • Alternate diagnoses mimicking vasculitis should be excluded prior to applying the criteria
Absolute requirement
Age ≥50 years at time of diagnosis
Additional clinical criteria
Morning stiffness in shoulders/neck +2
Sudden visual loss +3
Jaw or tongue claudication +2
New temporal headache +2
Scalp tenderness +2
Abnormal examination of the temporal artery* +2
Laboratory, imaging, and biopsy criteria
Maximum ESR ≥50 mm/hour or maximum CRP ≥10 mg/liter +3
Positive temporal artery biopsy or halo sign on temporal artery ultrasoundΔ +5
Bilateral axillary involvement +2
FDG-PET activity throughout aorta§ +2
Sum the scores for 10 items, if present. A score of ≥6 points is needed for the classification of giant cell arteritis.

ESR: erythrocyte sedimentation rate; CRP: C-reactive protein; FDG-PET: fluorodeoxyglucose positron emission tomography; DCVAS: Diagnostic and Classification Criteria in Vasculitis.

* Examination of the temporal artery showing absent or diminished pulse, tenderness, or hard "cord-like" appearance.

¶ Maximum ESR or CRP values prior to initiation of treatment for vasculitis.

Δ Presence of either definitive vasculitis on temporal artery biopsy or halo sign on temporal artery ultrasound. There are no specific histopathologic criteria to define definitive vasculitis on temporal artery biopsy. Presence of giant cells, mononuclear leukocyte infiltration, and fragmentation of the internal elastic lamina were independently associated with histopathologic interpretation of definitive vasculitis in the DCVAS cohort. Halo sign is defined by the presence of a homogenous, hypoechoic wall thickening on ultrasound.

◊ Bilateral axillary involvement is defined as luminal damage (stenosis, occlusion, or aneurysm) on angiography (computed tomography, magnetic resonance, or catheter-based) or ultrasound, halo sign on ultrasound, or FDG uptake on PET.

§ Abnormal FDG uptake in the arterial wall (eg, greater than liver uptake by visual inspection) through the descending thoracic and abdominal aorta on PET.
From: Ponte C, Grayson PC, Robson JC, et al. 2022 American College of Rheumatology/EULAR classification criteria for giant cell arteritis. Ann Rheum Dis 2022; 81:1647. Copyright © 2017 The Authors (or their employers). Reproduced with permission from BMJ Publishing Group Ltd.
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