What is the treatment for giant cell arteritis?

Treatment of Giant Cell Arteritis

Immediate Initial Treatment

Start high-dose oral glucocorticoids (prednisone 40-60 mg/day or 1 mg/kg/day, maximum 60 mg) immediately upon clinical suspicion of GCA, even before biopsy confirmation, to prevent irreversible vision loss. 1, 2

For Patients WITHOUT Visual Symptoms or Critical Ischemia

• Begin prednisone 40-60 mg daily (or 1 mg/kg/day, maximum 60 mg) as a single morning dose 1, 2
• Daily dosing is superior to alternate-day schedules for achieving remission 2
• Do not delay treatment while awaiting temporal artery biopsy—arrange biopsy within 2 weeks of starting steroids 2, 3

For Patients WITH Visual Symptoms or Threatened Vision Loss

• Administer intravenous methylprednisolone 500-1000 mg daily for 3 consecutive days immediately, followed by high-dose oral prednisone (1 mg/kg/day, maximum 60 mg) 1, 2
• This represents a neuro-ophthalmic emergency requiring immediate action 4
• The risk of vision loss in the second eye approaches 50% without prompt treatment 2, 3

First-Line Adjunctive Therapy

Add tocilizumab 162 mg subcutaneously weekly to glucocorticoids as first-line therapy for all newly diagnosed GCA patients, particularly those at high risk of steroid-related complications. 1, 2, 5

• Tocilizumab significantly reduces relapse rates and minimizes cumulative steroid exposure 1, 2
• The FDA has approved tocilizumab specifically for GCA treatment 5
• If tocilizumab is contraindicated (recurrent infections, history of gastrointestinal perforations, diverticulitis, or cost barriers), consider methotrexate as an alternative steroid-sparing agent 1, 2
• Glucocorticoids alone or glucocorticoids with methotrexate remain acceptable alternatives based on physician experience and patient preferences 1

Aspirin Prophylaxis

• Prescribe low-dose aspirin (75-150 mg/day) for all GCA patients unless contraindicated to protect against cardiovascular and cerebrovascular ischemic events 2

Glucocorticoid Tapering Protocol

Maintain the initial high-dose glucocorticoids for approximately 1 month until symptoms resolve and inflammatory markers (ESR, CRP) normalize, then begin gradual tapering. 1, 2, 3

Specific Tapering Targets

• Taper to 15-20 mg/day within 2-3 months 1, 2
• Taper to ≤5 mg/day after 1 year 1, 2
• Guide tapering by clinical symptoms AND normalization of ESR/CRP—do not rely on inflammatory markers alone 2, 6
• Most patients require approximately 2 years or more before glucocorticoids can be discontinued 1

Critical Tapering Caveat

• Avoid rapid steroid withdrawal below 5 mg/day, as this significantly increases relapse risk 1
• Rapid taper protocols (26-week schedules) show higher relapse rates compared to standard 52-week tapers 1
• Only 7% of patients can successfully discontinue therapy entirely while maintaining stable disease 6

Management of Disease Relapses

For Relapses WITH Cranial Ischemic Symptoms

• Add a non-glucocorticoid immunosuppressive agent (preferably tocilizumab) AND increase glucocorticoid dose to 40-60 mg/day 1, 2
• Tocilizumab is preferred over methotrexate for relapsing disease with cranial ischemia 2

For Relapses WITH Polymyalgia Rheumatica Symptoms Only

• Increase glucocorticoid dose by 5-15 mg/day above the last effective dose 1, 2
• Adding immunosuppressive agents may not be necessary 2

For Elevated Inflammatory Markers WITHOUT Clinical Symptoms

• Clinical observation and monitoring without escalation of immunosuppressive therapy is recommended 2, 3
• Do not treat laboratory values alone 2

Special Considerations for Large Vessel Involvement

• Obtain noninvasive vascular imaging (CT or MRI of thoracic aorta and branch vessels) to evaluate large vessel involvement in all newly diagnosed GCA patients 2
• For active extracranial large vessel involvement, combine oral glucocorticoids with a non-glucocorticoid immunosuppressive agent (tocilizumab preferred) rather than glucocorticoids alone 1, 2
• For severe GCA with worsening limb/organ ischemia, escalate immunosuppressive therapy before considering surgical intervention 2
• Immediate surgical intervention is reserved only for aortic aneurysms at high rupture risk or impending tissue/organ infarction 2

Monitoring Strategy

• Monitor ESR and CRP regularly to guide treatment decisions 1, 2, 6
• Implement long-term clinical monitoring for all patients, even those in apparent remission, to detect relapses 2, 3
• Provide osteoporosis prophylaxis (calcium, vitamin D, bisphosphonates as indicated) for all patients on prolonged glucocorticoid therapy 4, 7
• Monitor for glucocorticoid-related adverse effects including infections, hyperglycemia, hypertension, and weight gain 1, 2

Critical Pitfalls to Avoid

• Never delay treatment while awaiting biopsy confirmation—vision loss is often irreversible and occurs primarily before treatment initiation 2, 3, 6
• Do not rely solely on systemic symptoms to guide tapering; ESR and CRP are more reliable parameters 6
• Avoid rapid steroid tapers designed for clinical trials in real-world practice unless the patient is on tocilizumab 1
• Do not use infliximab for GCA, as it is associated with recurrent ocular symptoms and disease activity 2
• Do not use statins specifically for GCA treatment (though they may be appropriate for cardiovascular risk reduction) 1

Evidence Quality Note

The strongest evidence comes from the 2021 ACR/Vasculitis Foundation guideline 1 and 2018 EULAR recommendations 1, with the tocilizumab recommendation supported by high-quality RCT data demonstrating significant glucocorticoid-sparing effects and reduced relapse rates. The IV methylprednisolone recommendation for visual symptoms is based on lower-quality retrospective data, but the potential benefit in preventing irreversible blindness justifies its use 1.