What is the treatment for giant cell arteritis?

Treatment of Giant Cell Arteritis

Initiate high-dose glucocorticoids immediately upon clinical suspicion of GCA—even before diagnostic confirmation—to prevent irreversible vision loss and other ischemic complications, and strongly consider adding tocilizumab as first-line therapy to reduce relapse rates and minimize steroid exposure. 1, 2

Immediate Initial Management

Glucocorticoid Therapy

• Start treatment urgently without waiting for biopsy confirmation, as GCA is a medical emergency with imminent risk of permanent vision loss 1, 2
• For patients without threatened vision loss: Begin high-dose oral prednisone at 1 mg/kg/day (maximum 60 mg/day) 1, 2
• For patients with threatened vision loss or active visual symptoms: Administer intravenous methylprednisolone 500-1000 mg/day for 3 days, followed by high-dose oral prednisone 2
• Use daily dosing rather than alternate-day schedules to achieve higher remission rates 1, 2

The urgency cannot be overstated: without treatment, if one eye is affected, the risk of losing vision in the second eye approaches 50% 1. Visual loss occurs in 15-35% of patients, primarily before treatment initiation 1, 2.

First-Line Adjunctive Therapy

Tocilizumab

• Add tocilizumab to glucocorticoids as first-line combination therapy, particularly for patients at high risk of steroid-related complications 1, 2
• Tocilizumab is FDA-approved for GCA treatment and significantly reduces glucocorticoid requirements and flare rates 1, 3
• This combination approach is preferred by the American College of Rheumatology to improve outcomes and reduce relapse rates 1

Aspirin

• Prescribe low-dose aspirin (75-150 mg/day) for all patients unless contraindicated 2
• Aspirin is specifically recommended for patients with critical or flow-limiting involvement of vertebral or carotid arteries 1

Alternative Steroid-Sparing Agents

• Consider methotrexate as an alternative if tocilizumab is contraindicated or unavailable 2
• Methotrexate has moderate evidence supporting its use as a steroid-sparing agent 2

Glucocorticoid Tapering Strategy

Maintain initial high-dose glucocorticoids for approximately one month before beginning taper 1, 2. This approach is critical—avoid rapid steroid withdrawal as it leads to disease exacerbation 2.

Tapering Timeline

• At 3 months: Target dose of 10-15 mg/day 1, 2
• At 1 year: Target dose of ≤5 mg/day 2
• Guide tapering by clinical symptoms and normalization of inflammatory markers (ESR, CRP) 1, 2

Be aware that up to 60% of patients will experience one or more relapses during glucocorticoid tapering 1. This high relapse rate underscores the importance of close monitoring and consideration of steroid-sparing agents.

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
• This represents a medical emergency requiring aggressive treatment escalation 2

Relapse with Polymyalgia Rheumatica Symptoms Only

• Increasing glucocorticoid dose alone may be sufficient 2
• PMR symptoms often herald GCA relapse and require careful monitoring 1

Isolated Inflammatory Marker Elevation Without Symptoms

• Clinical observation and monitoring without escalation of immunosuppressive therapy is recommended 2
• Do not reflexively increase treatment based solely on laboratory values 2

Treatment Targets and Monitoring

Primary Treatment Target

• Achieve and maintain remission, defined as absence of clinical symptoms and systemic inflammation 4, 1
• Prevent tissue ischemia and vascular damage as co-primary goals 4

Monitoring Strategy

• Monitor disease activity using clinical symptoms and laboratory markers (ESR, CRP) regularly 1, 2
• Obtain baseline noninvasive vascular imaging to evaluate large vessel involvement 1
• Implement long-term clinical monitoring even in apparent remission to detect relapses 2

Special Considerations for Large Vessel Involvement

• For patients with active extracranial large vessel involvement: Use oral glucocorticoids combined with a non-glucocorticoid immunosuppressive agent rather than glucocorticoids alone 2
• For patients with severe GCA and worsening limb/organ ischemia: Escalate immunosuppressive therapy over immediate surgical intervention 2
• Consider immediate surgical intervention only for: Aortic aneurysms at high risk for rupture and impending/progressive tissue or organ infarction 2

Glucocorticoid-Related Complications Management

• Provide bone protection therapy to all patients on long-term glucocorticoids 1
• Consider prophylaxis for other steroid-related complications including hypertension, diabetes, peptic ulcer disease, and infection 5
• The high burden of glucocorticoid toxicity in elderly patients makes steroid-sparing agents like tocilizumab particularly valuable 1, 2

Critical Pitfalls to Avoid

• Never delay treatment while awaiting biopsy results—vision loss can be permanent and occurs rapidly 2, 6
• Do not use infliximab for GCA—it is associated with recurrent ocular symptoms and disease activity 2
• Avoid rapid steroid withdrawal—this leads to disease exacerbation 2
• Do not dismiss atypical presentations—GCA can present without headache, and 70% of such patients in one series developed permanent visual impairment 6
• Recognize that diagnostic delays are common—mean time from symptom onset to diagnosis averages 35 days, contributing to irreversible complications 6