Giant Cell Arteritis

Not Verified
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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).

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

Sources

No data available

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