Large-Vessel Vasculitis Classification: Draft Criteria Presented at ACR/ARHP 2018

Takayasu arteritis
Takayasu arteritis
The ACR and EULAR have developed draft classification criteria for giant cell arteritis and Takayasu arteritis.

The following article is part of conference coverage from the 2018 American College of Rheumatology and Association of Rheumatology Health Professionals (ACR/ARHP) Annual Meeting in Chicago, Illinois. Rheumatology Advisor’s staff will be reporting breaking news associated with research conducted by leading experts in rheumatology. Check back for the latest news from ACR/ARHP 2018 .

CHICAGO – The American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR) have collaborated to develop draft classification criteria for large-vessel vasculitis. Investigators presented the draft criteria at the 2018 ACR/ARHP Annual Meeting, held October 19-24, in Chicago, Illinois.

The new criteria, once approved, would update the 1990 classification criteria for giant cell arteritis and Takayasu arteritis.2,3 Recent imaging techniques, such as angiography, vascular ultrasound, and positron emission tomography are now widely used in clinical practice to diagnose large-vessel vasculitis. The updated criteria will reflect the critical role of vascular imaging in the diagnosis and management of large-vessel vasculitis.

Draft Classification Criteria for Giant Cell Arteritis

According to the proposed draft criteria, patients must have a diagnosis of vasculitis and be ≥40 years at the time of diagnosis.

In addition, patients must have ≥6 points of inclusion criteria to meet the threshold for classification. These criteria include clinical features (2 points each) such as morning stiffness in shoulders or neck, sudden visual loss, jaw or tongue claudication, new temporal headache, and scalp tenderness.

Additional areas of criteria include temporal artery exam findings, such as reduced pulse or tenderness (1 point), erythrocyte sedimentation rate ≥50 mm/h or C-reactive protein ≥10 mg/L (3 points), temporal artery biopsy (5 points for definite vasculitis; 2 points for possible vasculitis). Imaging finding criteria include temporal artery halo sign (5 points), bilateral axillary involvement (3 points), and fluorodeoxyglucose-positron emission tomography (FDG-PET) activity throughout aorta (3 points).

Draft Classification Criteria for Takayasu Arteritis

To be considered for the proposed Takayasu arteritis classification, patients must have a diagnosis of vasculitis, be ≤60 years at diagnosis, and have evidence of vasculitis on imaging.

Patients must also have ≥5 points of inclusion criteria to meet the threshold for classification. Clinical features include female sex (1 point), angina or ischemic cardiac pain attributable to vasculitis (2 points), and arm or leg claudication (2 points). Additional vascular exam finding criteria include arterial bruit (2 points), reduced pulse in upper extremity (2 points), reduced pulse or tenderness in the carotid artery (2 points), and systolic blood pressure difference in arms ≥20 mmHg (1 point).

Criteria for Takayasu arteritis also include angiographic and ultrasound findings, including the number of affected arteries (1 point for 1 artery, 2 points for 2 arteries, and 3 points for ≥3 arteries), vasculitis affecting paired branch arteries (1 point), and abdominal aorta involvement with renal or mesenteric involvement (3 points).

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“The prior ACR classification criteria were extremely important, but were published over 25 years ago, and both the practice of medicine and the methodology for creating such criteria have changed,” stated Peter A. Merkel, MD, MPH, chief of the Division of Rheumatology, and professor of medicine and epidemiology at the University of Pennsylvania.4

“The new criteria will take into consideration the advances in diagnostic imaging and other aspects of the evaluation of patients with vasculitis. The new data is generated from a large dataset derived from work in multiple countries. The intended result is a set of criteria more useful to current researchers and applicable to patients throughout the world.”

Peter C. Grayson, MD, of the National Institute of Arthritis, Musculoskeletal and Skin Disease, US National Institutes of Health, told attendees that age is an “almost perfect” predictor of classification for large-vessel vasculitis. Patients with giant cell arteritis are generally older than 60 years at diagnosis, while the population of those with Takayasu arteritis at diagnosis is usually younger than 40 years. However, according to the current 1990 classification criteria, there is a gray area for patients between 40 and 60 years.

Moving forward, the investigators aim to optimize the point based system after a review of cases who fail to meet either criteria for giant cell arteritis or Takayasu arteritis, or who meet both criteria simultaneously.

The investigators note that the draft criteria have not been approved by the ACR and are not yet ready for use.

For more coverage of ACR/ARHP 2018, click here.


  1. Merkel PA, Unizony S, Ponte C, Grayson PC, Suppiah P. ACR: Updated classification criteria for large-vessel vasculitis. Presented at: 2018 ACR/ARHP Annual Meeting; October 19-24, 2018; Chicago, IL. Session 5T116.
  2. Hunder GG, Bloch DA, Michel BA, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis.  Arthritis Rheum. 1990;33(8):1122-1128. 
  3. Arend WP, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. 1990;33(8):1129-1134. 
  4. ACR and EULAR present drafts of new classification criteria for large-vessel vasculitis [press release]. Chicago, IL: American College of Rheumatology. Published October 20, 2018. Accessed October 20, 2018.

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