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    Giant Cell Arteritis

    Inflammatory

    Last Updated Jan 23, 2024
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    First 5 Minutes

    Consider in all patients presenting with headache greater than 60 years of age.

    Context

    • Most common systemic vasculitis affecting large and medium sized arteries.
    • Very rare in those <50 years old. Peak incidence in seventh decade.
    • Can present with cranial and extracranial manifestations, complicating diagnosis.
    • Often co-occurs with polymyalgia rheumatica.
    • Visual loss or is the blindness most serious complication if left untreated.
    • Development of aneurysms, also a serious, less commonly seen complication.

    Diagnostic Process

    • American College of Rheumatology (ACR) developed classification criteria most widely used.
    • Includes clinical, laboratory, imaging, and biopsy data for scoring and risk stratification.
    • ACR guidelines mainly used for identifying patients with large vessel disease, and not necessarily targeted at clinical diagnosis.
    • Giant Cell Arteritis (GCA) should be suspected in those >50 with the following features:
      • Headache, especially unilateral.
      • Visual changes.
      • Jaw claudication.
      • Constitutional symptoms including fevers, fatigue, joint/muscle aches.
      • Elevated inflammatory markers (CRP, ESR).
    • Suspected GCA should be confirmed by temporal artery biopsy >1 cm or temporal artery Doppler ultrasound.

    Pitfalls

    • Sensitivity of temporal artery biopsy ranges from 50-95%.
    • High false negative rate with unilateral biopsy.
    • Glucocorticoids initiated prior to biopsy could reduce sensitivity of biopsy.

    Recommended Treatment

    • Those with no visual loss at diagnosis should be treated with high dose glucocorticoids.
      • Prednisone 40-60mg oral per day.
    • Tocilizumab should be added in addition to glucocorticoids at a dose of 162 mg subcutaneous weekly injection. Treatment duration generally 12-18 months, but should be left up to rheumatology recommendation.
    • Those with visual loss or cerebrovascular manifestations should be treated with intravenous glucocorticoids.
      • Methylprednisolone 500-1000 mg daily x 3 days followed by prednisone 40-60 mg oral daily.
    • Select cases with critical stenosis of affected vessels including cerebral or carotid arteries should have low dose aspirin added to treatment.
    • Typical tapering of glucocorticoids should be done after 2-4 weeks in most cases.

    Criteria For Hospital Admission

    • Most patients are suitable for outpatient management.
    • Patients with unstable vital signs or signs of vascular complication including aortic aneurysm or critical narrowing of cerebral or carotid arteries should be admitted.

    Criteria For Transfer To Another Facility

    Transport considerations

    • Patients may require referral to another centre if services such as rheumatology and vascular surgery are not available.

    Criteria For Close Observation And/or Consult

    • Patients should be referred to a vascular surgeon for temporal artery biopsy within one week.
    • Rheumatology should be consulted for outpatient follow-up.

    Criteria For Safe Discharge Home

    • Those that do not experience improvement in their symptoms within 72-96 hours should have close follow up.

    Quality Of Evidence?

    Justification

    For patients with new diagnosis of GCA, recommendation of adding tocilizumab over glucocorticoids alone.

    Two randomized trials have shown improved remission when tocilizumab used in conjunction with glucocorticoids.

    Moderate

    Recommendation of adding aspirin to patients with new diagnosis of GCA and critical flow-limiting involvement of the vertebral or carotid arteries

    Low

    Related Information

    Reference List

    1. Ameer MA, Peterfy RJ, Khazaeni B. Giant Cell Arteritis (Temporal Arteritis). In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 [cited 2023 Dec 13]. Available from: http://www.ncbi.nlm.nih.gov/books/NBK459376/


    2. Maz M, Chung SA, Abril A, Langford CA, Gorelik M, Guyatt G, et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis. Arthritis & Rheumatology. 2021 Aug;73(8):1349–65.


    3. Diagnosis of giant cell arteritis – UpToDate [Internet]. [cited 2023 Dec 13]. Available from: https://www.uptodate.com/contents/diagnosis-of-giant-cell-arteritis


    4. Smith JH, Swanson JW. Giant Cell Arteritis. Headache: The Journal of Head and Face Pain. 2014;54(8):1273–89.


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