Giant Cell Arteritis

Not yet reviewed

This information is AI generated and has not yet been reviewed by a specialist physician. AI can make mistakes.

Disease overview

Granulomatous inflammatory vasculitis of medium-to-large arteries with a preference for the temporal artery and other cranial arteries; cause is unknown but theroized to be autoimmune

Common symptoms

Classically presents >50 years old with new-onset headachetenderness over the temporal arteryjaw claudicationand artery prominence. May have amaurosis fugax and diplopia. Commonly causes fever and polymyalgia rheumatica in the neckshouldersand hips.

When to suspect

  • Recommendation 1

    No one specific test

  • Recommendation 2

    the American College of Rheumatology recommends at least 6 "points" from the following signs

  • Recommendation 3

    positive temporal artery biopsy or temporal artery halo sign on ultrasound (+5)

  • Recommendation 4

    ESR ≥50 mm/hour or CRP ≥10 mg/liter (+3)

  • Recommendation 5

    sudden visual loss (+3)

  • Recommendation 6

    morning stiffness, jaw or tongue claudication, new temporal headache, scalp tenderness, temporal artery abnormality, bilateral axillary involvement on imaging, and aortic FDG-PET activity (+2 each).

How to test

  • Recommendation 1

    No one specific test

  • Recommendation 2

    the American College of Rheumatology recommends at least 6 "points" from the following signs

  • Recommendation 3

    positive temporal artery biopsy or temporal artery halo sign on ultrasound (+5)

  • Recommendation 4

    ESR ≥50 mm/hour or CRP ≥10 mg/liter (+3)

  • Recommendation 5

    sudden visual loss (+3)

  • Recommendation 6

    morning stiffness, jaw or tongue claudication, new temporal headache, scalp tenderness, temporal artery abnormality, bilateral axillary involvement on imaging, and aortic FDG-PET activity (+2 each).

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Treatment

  • Recommendation 1

    Immediate corticosteroid therapy is required before any confirmatory testing upon suspicion of disease, to minimize risk of permanent vision loss. IV methylprednisolone is warranted for patients with existing visual/cranial symptoms before tapering on oral therapy.

Primary care

  • Recommendation 1

    Many patients require ongoing maintenance therapy to prevent relapses/flares. Consider bisphosphonates if prolonged steroid therapy is needed. DMARDs (specifically methotrexate, tocilizumab) can also be used instead of steroids for maintenance.

Further support

  • Recommendation 1

    Patients should be referred to rheumatology. The Vasculitis Foundation maintains a database of nationwide and international rheumatology clinics.Referral to Medical Genetics Department, if available. Initial virtual care is also available through organizations like TeleRare Health.

Clinical trials

Clinical Trials

Sources

No data available

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