What is the treatment for giant cell arteritis?

Treatment of Giant Cell Arteritis

The first-line treatment for giant cell arteritis (GCA) is high-dose oral glucocorticoids, with the addition of tocilizumab recommended as a steroid-sparing agent to reduce cumulative steroid exposure and minimize adverse effects. 1, 2

Initial Treatment Approach

• Start high-dose oral glucocorticoids (prednisone 1 mg/kg/day, maximum 60 mg/day) immediately upon clinical suspicion of GCA, even before biopsy confirmation, to prevent vision loss and other ischemic complications 1, 2
• Daily dosing of glucocorticoids is recommended over alternate-day schedules to achieve higher remission rates 1
• For patients with threatened vision loss, administer intravenous pulse methylprednisolone (500-1000 mg/day for 3 days) immediately, followed by high-dose oral prednisone 1, 3
• Arrange temporal artery biopsy as soon as possible, ideally within 2 weeks of starting glucocorticoids, to confirm diagnosis 2

Adjunctive Therapy

• Add tocilizumab to glucocorticoids as first-line therapy, especially for patients at high risk of steroid-related complications, to reduce relapse rates and minimize steroid exposure 1, 2, 4
• Consider methotrexate as an alternative steroid-sparing agent if tocilizumab is contraindicated 1, 2
• Prescribe low-dose aspirin (75-150 mg/day) for all patients with GCA unless contraindicated, to protect against cardiovascular and cerebrovascular events 1

Glucocorticoid Tapering

• Maintain initial high-dose glucocorticoids for approximately one month to ensure adequate disease control 2
• Begin gradual taper after symptoms are controlled and inflammatory markers have normalized, aiming for 10-15 mg/day by 3 months, and ≤5 mg/day after 1 year 2
• Guide tapering by clinical symptoms and normalization of inflammatory markers (ESR, CRP) 1, 2

Management of Relapses

• For patients with disease relapse with symptoms of cranial ischemia, add a non-glucocorticoid immunosuppressive agent (preferably tocilizumab) and increase the dose of glucocorticoids 1
• For relapses with symptoms of polymyalgia rheumatica, increasing the dose of glucocorticoids alone may be sufficient 1
• For patients with increased inflammatory markers alone without clinical symptoms, clinical observation and monitoring without escalation of immunosuppressive therapy is recommended 1

Special Considerations for Large Vessel Involvement

• Obtain noninvasive vascular imaging to evaluate large vessel involvement in newly diagnosed GCA 1
• For patients with active extracranial large vessel involvement, oral glucocorticoids combined with a non-glucocorticoid immunosuppressive agent is recommended over oral glucocorticoids alone 1
• For patients with severe GCA and worsening signs of limb/organ ischemia, escalate immunosuppressive therapy over immediate surgical intervention 1
• Consider immediate surgical intervention for aortic aneurysms at high risk for rupture and impending/progressive tissue or organ infarction 1

Monitoring

• Implement long-term clinical monitoring for all patients, even those in apparent remission, to detect potential relapses 1
• Monitor inflammatory markers (ESR, CRP) regularly to guide treatment decisions 1, 5
• For patients with increased inflammatory markers alone, without clinical symptoms, more frequent clinical and/or radiographic assessments for active disease may be warranted, but escalation of therapy is not recommended 1

Prognosis and Complications

• Visual loss occurs in 15-35% of patients with GCA, primarily before initiation of therapy 2, 3
• Without treatment, the risk of vision loss in the second eye is as high as 50% if one eye is already affected 3
• Only about 4% of GCA patients with visual loss show improvement with high-dose steroid therapy 5
• Glucocorticoid-related adverse events are common with long-term therapy 2, 6

Common Pitfalls and Caveats

• Do not delay treatment while awaiting biopsy results, as vision loss can be permanent 2, 3
• Do not rely solely on systemic symptoms to guide tapering of glucocorticoids; use ESR and CRP levels 5
• Avoid rapid steroid withdrawal as it can lead to disease exacerbation 2
• Do not use infliximab for GCA as it is associated with recurrent ocular symptoms and markers of disease activity 1
• Do not underestimate the duration of treatment needed; many patients require 1-2 years of therapy, and some may need low-dose glucocorticoids for several years 6, 5