What is the recommended management for giant cell arteritis?

Management of Giant Cell Arteritis

The recommended management for giant cell arteritis (GCA) includes immediate initiation of high-dose oral glucocorticoids (40-60 mg/day prednisone or equivalent), with the addition of tocilizumab as a steroid-sparing agent in selected patients. 1

Initial Treatment

Glucocorticoid Therapy

• Initial dosing:
  • High-dose oral glucocorticoids (40-60 mg/day prednisone or 1 mg/kg/day, maximum 80 mg) should be started immediately upon clinical suspicion of GCA 1, 2
  • For patients with visual symptoms (e.g., amaurosis fugax, vision loss):
    • Consider IV methylprednisolone 500-1000 mg/day for 3 days before transitioning to oral therapy 1, 2
  • Daily dosing is preferred over alternate-day regimens 1, 3

Adjunctive Therapy

• Add aspirin (75-150 mg/day) for patients with critical or flow-limiting involvement of vertebral or carotid arteries to reduce ischemic complications 1, 2
• Consider tocilizumab (162 mg subcutaneously weekly) in combination with glucocorticoids for:
  • Patients at high risk for glucocorticoid-related adverse events
  • Refractory or relapsing disease
  • Patients with active extracranial large vessel involvement 1, 4
• Methotrexate may be used as an alternative to tocilizumab when tocilizumab is contraindicated 1

Diagnostic Testing

• Temporal artery biopsy should be performed within 2 weeks of starting glucocorticoids 1
  • Obtain a long-segment specimen (>1 cm)
  • Initially perform unilateral biopsy; bilateral may be considered if symptoms are not clearly localized
• Vascular imaging (ultrasound, MRI, CT angiography) should be considered in patients with suspected GCA and negative biopsy results 2

Glucocorticoid Tapering

1. Initial phase: Maintain high-dose glucocorticoids for 2-4 weeks until symptoms resolve and inflammatory markers normalize
2. Tapering phase:
   • Reduce to 15-20 mg/day within 2-3 months
   • Aim for ≤10 mg/day by 6 months
   • Target ≤5 mg/day after one year
   • Complete tapering based on clinical response and laboratory parameters 1

Management of Relapses

Minor Relapses (e.g., polymyalgia rheumatica symptoms)

• Increase glucocorticoid dose (5-15 mg/day above the last effective dose) 1

Major Relapses (e.g., cranial ischemic symptoms)

• Increase glucocorticoids to 40-60 mg/day 1
• Add tocilizumab (preferred) or methotrexate if not already on immunosuppressive therapy 1
• For patients already on glucocorticoids with cranial ischemic symptoms, adding tocilizumab is preferred over methotrexate 1

Monitoring

• Clinical monitoring is strongly recommended long-term, even for patients in apparent remission 1
  • Regular assessment of symptoms, physical examination
  • Monitor inflammatory markers (ESR, CRP)
  • Frequency depends on disease activity, medication regimen, and patient reliability
• For isolated increases in inflammatory markers without clinical symptoms:
  • Clinical observation and monitoring without escalation of therapy is recommended 1
  • Consider more frequent clinical/radiographic assessments

Surgical Considerations

• For patients requiring vascular surgical intervention, decisions should be made collaboratively between vascular surgeons and rheumatologists 1
• For patients with severe GCA and worsening limb/organ ischemia:
  • Escalate immunosuppressive therapy first
  • Consider surgical intervention for aortic aneurysms at high risk for rupture or impending tissue/organ infarction 1
• Use high-dose glucocorticoids during the periprocedural period for patients with active disease 1

Common Pitfalls and Caveats

1. Delaying treatment: Never delay glucocorticoid therapy while awaiting biopsy results when GCA is strongly suspected, as this increases risk of permanent vision loss
2. Inadequate initial dosing: Underdosing glucocorticoids initially may lead to irreversible ischemic complications
3. Too-rapid tapering: Overly aggressive glucocorticoid tapering often leads to disease relapse
4. Overreliance on inflammatory markers: Treatment decisions should not be based solely on inflammatory markers without clinical correlation
5. Failure to provide prophylaxis: Patients on long-term glucocorticoids should receive appropriate prophylaxis for osteoporosis and other steroid-related complications

The management of GCA requires balancing the risks of disease-related complications against the adverse effects of treatment. Long-term clinical monitoring is essential even after apparent remission due to the risk of relapse and vascular complications.