What is the treatment for temporal arteritis?

Treatment of Temporal Arteritis (Giant Cell Arteritis)

Start high-dose oral glucocorticoids (prednisone 1 mg/kg/day, maximum 60 mg/day) immediately upon clinical suspicion of temporal arteritis, even before biopsy confirmation, and add tocilizumab as first-line adjunctive therapy to reduce relapse rates and minimize steroid exposure. 1, 2, 3

Immediate Treatment Initiation

For Patients Without Visual Symptoms or Critical Cranial Ischemia

• Begin prednisone 1 mg/kg/day (maximum 60 mg/day) immediately upon clinical suspicion—do not wait for biopsy results, as delay can result in permanent vision loss 1, 2, 3
• Use daily dosing rather than alternate-day schedules to achieve higher remission rates 1, 3
• Add tocilizumab to glucocorticoids as first-line combination therapy, particularly for patients at high risk of steroid-related complications 1, 2, 3

For Patients With Threatened Vision Loss or Critical Cranial Ischemia

• Administer intravenous pulse methylprednisolone 500-1000 mg/day for 3 days immediately, followed by high-dose oral prednisone 1 mg/kg/day 2, 4, 3
• This aggressive approach is critical because without treatment, the risk of vision loss in the second eye reaches 50% if one eye is already affected 2, 3

Diagnostic Confirmation While on Treatment

• Arrange temporal artery biopsy as soon as possible, ideally within 2 weeks of starting glucocorticoids, as histopathologic changes remain detectable during this window 1, 2
• Obtain a long-segment biopsy specimen (>1 cm) to improve diagnostic yield, since temporal arteritis is a focal and segmental disease 1, 2
• Initially perform unilateral biopsy; proceed with contralateral biopsy only if the first is negative and clinical suspicion remains high 1
• If temporal artery biopsy is negative but clinical suspicion persists, obtain noninvasive vascular imaging to evaluate large vessel involvement 2, 3

Important caveat: In the United States, temporal artery biopsy remains preferred over ultrasound for diagnosis, as ultrasound is operator-dependent and less widely available with appropriate expertise compared to Europe 1

Adjunctive Steroid-Sparing Therapy

First-Line Adjunctive Agent

• Tocilizumab is the preferred steroid-sparing agent and should be added to glucocorticoids at treatment initiation, as it has demonstrated efficacy in reducing glucocorticoid requirements and flare rates up to 52 weeks 1, 2, 3
• Tocilizumab is FDA and EMA approved for temporal arteritis and is particularly beneficial for patients requiring prolonged glucocorticoid therapy or those with steroid-related adverse effects 1, 4

Alternative Steroid-Sparing Agent

• Consider methotrexate as an alternative if tocilizumab is contraindicated, though evidence supporting methotrexate is more modest, achieving only modest reduction in cumulative glucocorticoid dose 1, 2, 4

Cardiovascular Protection

• Prescribe low-dose aspirin 75-150 mg/day for all patients unless contraindicated, to protect against cardiovascular and cerebrovascular events 3

Glucocorticoid Tapering Strategy

• Maintain initial high-dose glucocorticoids for approximately one month to ensure adequate disease control 4, 3
• Begin gradual taper after symptoms are controlled and inflammatory markers (ESR, CRP) have normalized 1, 2, 4
• Target prednisone dose of 10-15 mg/day by 3 months and ≤5 mg/day after 1 year 2, 4
• Guide tapering by monitoring clinical symptoms (headache, scalp tenderness, jaw claudication, visual symptoms) and normalization of inflammatory markers 1, 2, 4, 3

Critical pitfall: Avoid rapid steroid withdrawal as it can lead to disease exacerbation and relapse 3

Management of Disease Relapse

Relapse With Cranial Ischemic Symptoms

• Add a non-glucocorticoid immunosuppressive agent (preferably tocilizumab over methotrexate) and increase glucocorticoid dose 1, 2, 4, 3
• Tocilizumab is preferred for relapsing disease with cranial symptoms due to superior efficacy 4, 3

Relapse With Polymyalgia Rheumatica Symptoms Only

• Increasing glucocorticoid dose alone may be sufficient for shoulder/hip girdle pain without cranial symptoms 3

Elevated Inflammatory Markers Without Clinical Symptoms

• Clinical observation and monitoring without escalation of immunosuppressive therapy is recommended, as isolated marker elevation does not necessarily indicate active disease 2, 3

Special Considerations for Large Vessel Involvement

• Obtain noninvasive vascular imaging at diagnosis to evaluate extracranial large vessel involvement 3
• For patients with active extracranial large vessel involvement, combine oral glucocorticoids with a non-glucocorticoid immunosuppressive agent rather than using glucocorticoids alone 2, 3
• For severe disease with worsening limb/organ ischemia, escalate immunosuppressive therapy before considering surgical intervention 3
• Consider immediate surgical intervention only for aortic aneurysms at high risk for rupture or impending/progressive tissue or organ infarction 3

Long-Term Monitoring

• Implement long-term clinical monitoring for all patients, even those in apparent remission, to detect relapses 2, 3
• Monitor for recurrence of headache, scalp tenderness, jaw claudication, and visual symptoms that may indicate relapse 4
• Monitor inflammatory markers (ESR, CRP) regularly to guide treatment decisions 4, 3
• Monitor for glucocorticoid-related adverse effects and provide appropriate prophylaxis, particularly bone protection 2

Important prognostic information: Visual loss occurs in 15-35% of patients with temporal arteritis, primarily before initiation of therapy, and glucocorticoid-related adverse events occur in 86% of patients with long-term therapy 2, 3

Agents to Avoid

• Do not use infliximab (TNF inhibitor) for temporal arteritis, as it is associated with recurrent ocular symptoms and markers of disease activity 3