What is the management of giant cell arteritis?

Management of Giant Cell Arteritis

Initial Treatment

For patients with suspected or confirmed giant cell arteritis (GCA), immediate initiation of high-dose glucocorticoids is strongly recommended as the cornerstone of therapy to prevent irreversible complications such as vision loss. 1, 2

Initial Glucocorticoid Therapy

• Dosing:
  • Oral prednisone: 40-60 mg/day (or equivalent) for uncomplicated GCA 1, 2
  • IV methylprednisolone: 500-1000 mg/day for 3 days for patients with visual symptoms or threatened vision 2, 3
• Do not delay treatment while awaiting biopsy results, as permanent vision loss occurs in 14-35% of untreated patients 2
• Initial high-dose therapy should be maintained until symptoms resolve and inflammatory markers normalize (typically 2-4 weeks) 1

Glucocorticoid Tapering

• After clinical response, begin gradual taper:
  • First month: Reduce by 5-10 mg every 1-2 weeks to 20 mg/day
  • Second and third months: Slower reduction to 10-15 mg/day
  • Beyond 3 months: Very slow taper (1 mg reduction every 1-2 months)
• Total treatment duration typically 1-2 years, though some patients require longer therapy 4
• Monitor for relapse during tapering (recurrent symptoms, elevated inflammatory markers)

Glucocorticoid-Sparing Therapy

Tocilizumab

• Add tocilizumab (162 mg subcutaneously weekly) to glucocorticoid therapy for:
  • Patients with relapsing disease while on moderate-to-high dose glucocorticoids 1
  • Patients at high risk for glucocorticoid-related adverse effects 5, 6
  • Patients requiring prolonged glucocorticoid therapy 1, 7
• FDA-approved for GCA and reduces glucocorticoid requirements and flare rates 2, 5

Alternative Immunosuppressive Agents

• Methotrexate may be considered when tocilizumab is contraindicated or unavailable 1, 6, 4
• Other agents (azathioprine, TNF inhibitors) have less evidence but may be considered in refractory cases 7

Adjunctive Therapy

• Aspirin: Add low-dose aspirin (75-150 mg/day) for patients with critical or flow-limiting involvement of vertebral or carotid arteries 1, 2
• Osteoporosis prophylaxis: Calcium, vitamin D, and bisphosphonates as appropriate 2
• Blood pressure and glucose monitoring for patients on glucocorticoid therapy

Monitoring

• Clinical monitoring is strongly recommended long-term even after apparent remission 1

• Regular assessment should include:

  • Symptoms (headache, visual disturbances, jaw claudication, PMR symptoms)
  • Physical examination (temporal artery tenderness, pulses)
  • Laboratory markers (ESR, CRP) every 4-8 weeks initially, then every 3 months 1
  • Vascular imaging for patients with large-vessel involvement 1

• Important: Increased inflammatory markers alone without clinical symptoms do not warrant escalation of therapy but should prompt more frequent monitoring 1

Management of Relapse

• For disease relapse with cranial ischemic symptoms:
  • Add a nonglucocorticoid immunosuppressive agent (preferably tocilizumab) and increase glucocorticoid dose rather than increasing glucocorticoids alone 1
• For relapse with only polymyalgia rheumatica symptoms:
  • Increasing glucocorticoid dose alone may be sufficient 1

Vascular Complications Management

• For patients with severe GCA and worsening signs of limb/organ ischemia:
  • Escalate immunosuppressive therapy rather than proceeding directly to surgical intervention 1
• For patients requiring vascular surgical intervention:
  • Use high-dose glucocorticoids during the periprocedural period if active disease is present 1
  • Surgical intervention should be a collaborative decision between vascular surgeon and rheumatologist 1

Common Pitfalls and Caveats

• Do not delay treatment while awaiting temporal artery biopsy results
• Do not taper glucocorticoids too rapidly, as this increases risk of relapse
• Do not rely solely on inflammatory markers to guide treatment decisions
• Do not discontinue monitoring after apparent remission, as late relapses can occur
• Consider glucocorticoid-related adverse effects (diabetes, osteoporosis, hypertension, infections) and implement appropriate preventive measures