What is the treatment for giant cell arteritis?

Treatment of Giant Cell Arteritis

Immediate Initial Management

Start high-dose glucocorticoids immediately upon clinical suspicion of GCA, even before biopsy confirmation, as this is a medical emergency with imminent risk of permanent vision loss. 1, 2

Standard Presentation (No Vision Threat)

• Begin oral prednisone 1 mg/kg/day (maximum 60 mg/day) immediately 1, 2
• Use daily dosing rather than alternate-day schedules for superior remission rates 1, 2
• Do not delay treatment while awaiting temporal artery biopsy results 2, 3

Vision-Threatening Presentation

• Administer intravenous methylprednisolone 500-1000 mg/day for 3 consecutive days 2
• Follow with high-dose oral prednisone as above 2
• Without treatment, if one eye is affected, the second eye has up to 50% risk of vision loss 1, 2

First-Line Adjunctive Therapy

Add tocilizumab to glucocorticoids as first-line combination therapy to significantly reduce relapse rates and cumulative steroid exposure. 1, 2

• Tocilizumab is FDA-approved for GCA treatment 4
• The American College of Rheumatology recommends tocilizumab in combination with glucocorticoids to reduce glucocorticoid requirements and flare rates 1
• Particularly important for patients at high risk of steroid-related complications 2
• If tocilizumab is contraindicated, consider methotrexate (10-15 mg/week) as an alternative steroid-sparing agent, though it has more modest efficacy 5, 2

Aspirin Therapy

• Prescribe low-dose aspirin 75-150 mg/day to all patients unless contraindicated 5, 1
• Aspirin protects against cardiovascular and cerebrovascular events 5
• Consider gastroduodenal mucosal protection when starting aspirin 5

Glucocorticoid Tapering Protocol

• Maintain initial high-dose glucocorticoids for approximately one month 1, 2
• Begin gradual taper only after symptoms are controlled and inflammatory markers (ESR, CRP) have normalized 1, 2
• Target dose of 10-15 mg/day by 3 months 1, 2
• Aim for ≤5 mg/day after 1 year 2
• Guide tapering decisions by clinical symptoms and inflammatory markers, not imaging alone 5, 1

Management of Disease Relapse

Relapse with Cranial Ischemic Symptoms

• Add a non-glucocorticoid immunosuppressive agent (tocilizumab preferred over methotrexate) 1, 2
• Increase glucocorticoid dose 1, 2

Relapse with Polymyalgia Rheumatica Symptoms Only

• Increasing glucocorticoid dose alone may be sufficient 2
• Up to 60% of patients experience one or more relapses during glucocorticoid tapering 1

Elevated Inflammatory Markers Without Symptoms

• Clinical observation and monitoring without escalation of immunosuppressive therapy is recommended 2
• ESR elevation typically accompanies true clinical relapse 5

Special Considerations for Large Vessel Involvement

• Obtain baseline noninvasive vascular imaging to evaluate large vessel involvement 1, 2
• For active extracranial large vessel involvement, use oral glucocorticoids combined with a non-glucocorticoid immunosuppressive agent rather than glucocorticoids alone 2
• For severe GCA with worsening limb/organ ischemia, escalate immunosuppressive therapy over immediate surgical intervention 2
• Consider immediate surgery only for aortic aneurysms at high risk for rupture or impending tissue/organ infarction 2

Monitoring Strategy

• Monitor disease activity using clinical symptoms and laboratory markers (ESR, CRP) 5, 1
• Implement long-term clinical monitoring for all patients, even those in apparent remission, to detect relapses 2
• Treatment target should be remission, defined as absence of clinical symptoms and systemic inflammation 1

Supportive Care

• Provide bone protection therapy to all patients on long-term glucocorticoids 1
• Consider prophylaxis against Pneumocystis jiroveci and osteoporosis 5

Critical Pitfalls to Avoid

• Never delay treatment while awaiting biopsy confirmation - vision loss can be permanent and occurs in 15-35% of patients, primarily before treatment initiation 1, 2, 3
• Do not use infliximab - it does not reduce relapse risk and is not recommended in GCA 5, 2
• Avoid rapid steroid withdrawal - this leads to disease exacerbation 2
• Do not rely on statins for disease modification - they do not influence clinical profile or glucocorticoid requirements 5

Multidisciplinary Coordination

• Rheumatologists should coordinate long-term management 6
• Immediate ophthalmology consultation is essential for any visual symptoms 6
• Involve vascular surgeons for peripheral limb ischemia complications 6
• Neurologists manage stroke and cerebral vessel complications 6