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Giant cell arteritis

Last updated: January 27, 2021

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Giant cell arteritis (GCA) is a type of autoimmune vasculitis that causes chronic inflammation of large and medium-sized arteries, in particular the carotid arteries, its major branches, and the aorta. GCA is most common in women over the age of 50 and of northern European descent, and approximately 50% of patients also have polymyalgia rheumatica. Patients usually present with constitutional symptoms (e.g., fever, weight loss, night sweats, fatigue, and malaise), new-onset headache, a tender, hardened temporal artery, jaw claudication, or amaurosis fugax. If there is strong clinical suspicion for GCA, glucocorticoids should be administered immediately, even prior to diagnostic workup if needed, to reduce the risk of permanent vision loss and cerebral ischemia. Laboratory studies typically show signs of inflammation (e.g., elevated erythrocyte sedimentation rate and CRP), and temporal artery biopsy and/or imaging (e.g., Duplex ultrasound) should be performed to confirm the diagnosis. The classic histopathological findings are mononuclear infiltration of the vessel wall and formation of giant cells. Patients should be referred to rheumatology for follow-up because most patients require long-term therapy with glucocorticoids (≥ 2 years).

  • Sex: > [1]
  • Peak incidence: 70–79 years (rarely seen in patients < 50 years) [2]

Women of advanced age are particularly prone to the disease.

Epidemiological data refers to the US, unless otherwise specified.

Giant cell arteritis is thought to be due to a cell-mediated immune response to endothelial injury. However, the initiating factors are not fully understood.

  1. Inflammation
  2. Local vascular damage
  3. Concentric intimal hyperplasia: macrophages and giant cells produce PDGF and VEGF stimulate intimal proliferation reduced blood flow and ischemia

About 50% of patients with giant cell arteritis also have polymyalgia rheumatica.

Demonstration of arterial inflammation on imaging and/or histopathology is required to make a diagnosis of GCA. In patients with features of cranial GCA, the ACR classification criteria for GCA can be used to differentiate GCA from other forms of vasculitis. [6][9][10]

If GCA is highly suspected, diagnostics should not delay the initiation of high-dose glucocorticoids to minimize the risk of vision loss. [11]

Laboratory studies [6][9]

Confirmatory diagnostic studies

All patients require imaging and/or a temporal artery biopsy to confirm the diagnosis. [6][10][14][15]

Temporal artery biopsy (gold standard)

  • Indications
    • Consider in all patients with suspected GCA [10]
    • High clinical suspicion for GCA despite inconclusive findings on imaging
  • Findings: See “Pathology.”
  • Important considerations

Do not delay therapy for TAB results in patients with a high clinical suspicion of GCA.

Temporal artery biopsy (TAB) with histopathology is the only test that can definitively confirm GCA and should be considered in all patients, especially if GCA cannot be ruled out with imaging. [10]

Duplex ultrasound (US) [18][19][20]

  • Indications
  • Supportive findings [10][18]
    • Edema and thickening of the vessel wall (halo sign)
    • Noncompressible artery (compression sign)
    • Stenosis and occlusion [22]
  • Disadvantages [20]
    • Operator-dependent
    • Less sensitive in detecting thoracic aortic involvement

Additional imaging studies [6][23][24]

The characteristic diagnostic findings in GCA are raised inflammatory markers in combination with evidence of vascular inflammation on imaging and/or histopathology.

Differential diagnoses of GCA

Clinical criteria to differentiate GCA from other forms of vasculitis [15][25]

These criteria were developed in 1990 by the American College of Rheumatology (ACR) to differentiate cranial GCA from other forms of vasculitis. They should not be used to diagnose cranial GCA, for which they have low sensitivity and low positive predictive value. [7][26][27]

ACR criteria for the classification of GCA [15][25]
Criteria Score
Patient features

Headache (new onset or different from previous headaches)

1
Aged ≥ 50 years at disease onset 1
Examination findings Abnormalities of the temporal artery (decreased pulsation or tenderness to palpation) 1
Diagnostics

Histopathological abnormalities in the temporal artery

1
Elevated ESR (≥ 50 mm/h) 1

Interpretation
Score ≥ 3
: GCA is likely; investigate as needed (see “Diagnostics” section above).
Score < 3: Consider alternative diagnoses (see “Differential diagnoses of GCA” above).

The differential diagnoses listed here are not exhaustive.

Approach [6]

  • Initial high-dose glucocorticoid therapy for 2–4 weeks is indicated for all patients with newly diagnosed GCA or recurrent GCA symptoms.
  • Consults
    • Rheumatology consult for all patients
    • Urgent specialist consults as needed for complications [28][29]
      • Visual symptoms: ophthalmology consult
      • Neurological symptoms: neurology consult
      • Large-vessel involvement: vascular surgery consult
  • After the completion of high-dose therapy, taper the dose of glucocorticoid therapy to a maintenance dose.

Glucocorticoid therapy [6][14][15]

This is the mainstay of treatment for patients with GCA.

Initial high-dose therapy (induction therapy)

Immediate administration of high-dose glucocorticoids is crucial to lowering the risk of permanent vision loss in patients with giant cell arteritis.

Maintenance therapy [6][14][21]

  • Initiate after acute symptoms have resolved.
  • Slowly taper glucocorticoids to the lowest dose needed to control symptoms. [6]
  • Length of treatment: no consensus; generally ≥ 2 years [14]
  • Administer prophylactic measures against complications of long-term glucocorticoid therapy as needed.

Adjunct therapy

  • Glucocorticoid-sparing therapy [14][21][30]
  • Low-dose aspirin
    • Not routinely recommended
    • Consider in patients with preexisiting cardiovascular or cerebrovascular disease [14]

Begin treatment as soon as GCA is suspected. Do not wait for a biopsy.

We list the most important complications. The selection is not exhaustive.

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