What is the treatment for Giant Cell Arteritis (GCA)?

Treatment of Giant Cell Arteritis

Immediate Initial Management

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

For Patients WITHOUT Visual Symptoms or Critical Cranial Ischemia

• Initiate high-dose oral glucocorticoids (prednisone 40-60 mg/day) rather than IV pulse methylprednisolone as first-line therapy 1
• The routine use of IV pulse glucocorticoids in this population may increase infection risk without clear benefit, particularly in elderly patients 1
• Daily dosing is superior to alternate-day schedules for achieving remission 2

For Patients WITH Visual Symptoms or Critical Cranial Ischemia

• Administer IV pulse methylprednisolone (500-1000 mg/day for 3 consecutive days) immediately, followed by high-dose oral prednisone 2, 3
• This represents a neuro-ophthalmic emergency requiring immediate intervention to prevent permanent vision loss 2, 3
• Visual loss occurs in 15-35% of GCA patients, primarily before treatment initiation, and without treatment the risk of second-eye involvement approaches 50% 2

Glucocorticoid-Sparing Therapy

Add tocilizumab to glucocorticoids as first-line combination therapy, particularly for patients at high risk of steroid-related complications. 1, 2

Tocilizumab (FDA-Approved for GCA)

• Tocilizumab is FDA-approved for treatment of GCA in adult patients 4
• It demonstrates significant glucocorticoid-sparing effects, reducing relapse rates and minimizing steroid exposure 1
• The subcutaneous formulation provides the best-quantified glucocorticoid-sparing effect, though IV formulation is also effective 1
• Consider tocilizumab especially for patients with active extracranial large vessel involvement 1

Alternative Glucocorticoid-Sparing Agents

• Methotrexate can be considered as an alternative if tocilizumab is contraindicated due to recurrent infections, history of gastrointestinal perforations/diverticulitis, or cost concerns 1
• The glucocorticoid-sparing effect of methotrexate is smaller than tocilizumab, but it may decrease relapse risk and reduce cumulative steroid exposure 1, 5
• Abatacept can be considered if tocilizumab and methotrexate are not effective 1

Agents NOT Recommended

• Do not use statins specifically for GCA treatment, as they provide no clinically significant immunosuppressive effect for GCA 1
• Do not use infliximab for GCA, as it is associated with recurrent ocular symptoms and markers of disease activity 2

Aspirin Therapy

Prescribe low-dose aspirin (75-150 mg/day) for all GCA patients unless contraindicated to protect against cardiovascular and cerebrovascular events. 2, 6

• For patients with critical or flow-limiting involvement of vertebral or carotid arteries, adding aspirin is conditionally recommended 1

Glucocorticoid Tapering Strategy

Maintain initial high-dose glucocorticoids for approximately one month to ensure adequate disease control, then begin gradual taper. 2

Tapering Schedule

• Aim for prednisone 10-15 mg/day by 3 months 2
• Target ≤5 mg/day after 1 year 2
• Guide tapering by clinical symptoms and normalization of inflammatory markers (ESR, CRP) 2
• Most patients require 1-2 years of treatment, though some may need low-dose glucocorticoids for several years 6, 3

Important Caveat

• Avoid rapid steroid withdrawal as it leads to disease exacerbation 2
• The optimal duration should be individualized based on clinical manifestations, glucocorticoid toxicity, number of flares, and patient preferences 1

Management of Disease Relapse

Relapse WITH Cranial Ischemic Symptoms

Add tocilizumab (preferred over methotrexate) and increase glucocorticoid dose rather than increasing glucocorticoids alone. 1, 2

• Tocilizumab demonstrates superior glucocorticoid-sparing effects compared to methotrexate in comparative analyses 1
• Methotrexate remains an option for patients unable to tolerate or access tocilizumab 1

Relapse WITH Polymyalgia Rheumatica Symptoms Only

• Increasing the glucocorticoid dose alone may be sufficient for PMR symptoms without cranial involvement 1, 2

Elevated Inflammatory Markers WITHOUT Clinical Symptoms

Implement clinical observation and monitoring without escalating immunosuppressive therapy. 1, 2

• Increases in ESR and CRP can be non-specific and do not warrant treatment escalation in the absence of clinical disease activity 1
• However, increased inflammatory markers may warrant more frequent clinical and/or radiographic assessments 1

Management of Large Vessel Involvement

Obtain noninvasive vascular imaging (MR or CT angiography) to evaluate large vessel involvement in all newly diagnosed GCA patients. 1, 2

Active Extracranial Large Vessel Disease

• Use oral glucocorticoids combined with a non-glucocorticoid immunosuppressive agent (tocilizumab or methotrexate) rather than glucocorticoids alone 1, 2

Severe Limb/Organ Ischemia

• Escalate immunosuppressive therapy first rather than immediate surgical intervention, as patients can develop collateral vessels 1, 2
• Consider immediate surgical intervention only for: 1, 2
  • Aortic aneurysms at high risk for rupture
  • Impending/progressive tissue or organ infarction or damage

Long-Term Monitoring

Implement long-term clinical monitoring for all patients, even those in apparent remission, to detect potential relapses. 1, 2

• This is a strong recommendation given minimal risks and potential catastrophic outcomes without monitoring 1
• Monitor inflammatory markers (ESR, CRP) regularly to guide treatment decisions 2
• Frequency depends on duration of remission, site of involvement, risk of disease progression, and whether patient is receiving immunosuppressive therapy 1

Supportive Care

• Prescribe calcium, vitamin D, and peptic ulcer prophylaxis as indicated with glucocorticoid therapy 3
• Implement osteoporosis prophylaxis regularly 7

Critical Pitfalls to Avoid

• Never delay treatment while awaiting biopsy results, as vision loss can be permanent and irreversible 2, 3
• Temporal artery biopsy should be performed after treatment initiation to confirm diagnosis 3
• Glucocorticoid-related adverse events are common with long-term therapy and require proactive management 1, 6