What is the recommended management for a patient with giant cell arteritis?

Management of Giant Cell Arteritis

Initial Treatment: Start Glucocorticoids Immediately

For patients with suspected giant cell arteritis, initiate high-dose oral prednisone (1 mg/kg/day, maximum 60 mg/day) immediately upon clinical suspicion—do not wait for biopsy confirmation. 1, 2, 3

Treatment Algorithm Based on Clinical Presentation

Without visual symptoms or cranial ischemia:

• Start high-dose oral prednisone 1 mg/kg/day (maximum 60 mg/day) as a single morning dose 1, 2, 3
• Daily dosing is superior to alternate-day schedules for achieving remission 1, 4

With threatened vision loss or acute visual symptoms:

• Administer IV methylprednisolone 500-1000 mg/day for 3 consecutive days immediately 1, 2, 3, 5
• Follow with high-dose oral prednisone 1 mg/kg/day (maximum 60 mg/day) 1, 2, 3, 5
• This is a neuro-ophthalmic emergency requiring immediate treatment 5, 6

Add Tocilizumab as First-Line Therapy

Combine oral glucocorticoids with tocilizumab from the start, particularly for patients at high risk of steroid complications. 1, 2, 3

• Tocilizumab reduces relapse rates and minimizes cumulative steroid exposure 1, 2, 3
• Consider methotrexate as an alternative steroid-sparing agent only if tocilizumab is contraindicated 2, 3, 7, 8
• For extracranial large vessel involvement, glucocorticoids combined with a non-glucocorticoid immunosuppressive agent is recommended over glucocorticoids alone 1

Diagnostic Confirmation While on Treatment

Arrange temporal artery biopsy within 2 weeks of starting glucocorticoids—the biopsy remains diagnostic even after treatment initiation. 2, 3

• Obtain a long-segment biopsy (>1 cm) to improve diagnostic yield 2
• If biopsy is negative but clinical suspicion remains high, obtain noninvasive vascular imaging (CT/MRI angiography, ultrasound, or FDG-PET) to evaluate for large vessel involvement 1, 2, 3

Glucocorticoid Tapering Strategy

Maintain initial high-dose glucocorticoids for approximately 1 month until symptoms resolve and inflammatory markers normalize. 2, 3, 9

• Begin gradual taper after disease control is achieved, aiming for 10-15 mg/day by 3 months 2, 3
• Target ≤5 mg/day after 1 year 2, 3
• Guide tapering by clinical symptoms and normalization of ESR/CRP, not by inflammatory markers alone 1, 2, 3
• Most patients require 1-2 years of therapy to avoid relapse 5, 7

Management of Disease Relapse

For relapse with cranial ischemic symptoms, add tocilizumab (preferred over methotrexate) and increase glucocorticoid dose. 1, 3, 9

• For relapse while receiving moderate-to-high dose glucocorticoids, add a non-glucocorticoid immunosuppressive agent 1
• For isolated elevation of inflammatory markers without clinical symptoms, observe and monitor without escalating therapy 1, 2, 3

Adjunctive Therapies

Prescribe low-dose aspirin (75-150 mg/day) for all patients unless contraindicated to reduce cranial ischemic complications. 3, 7, 8, 6

• Add aspirin for patients with critical or flow-limiting involvement of vertebral or carotid arteries 1
• Provide bone protection (calcium, vitamin D, bisphosphonates as indicated) for all patients on long-term glucocorticoids 5, 8
• Consider peptic ulcer prophylaxis 5

Long-Term Monitoring

Implement long-term clinical monitoring for all patients, even those in apparent remission, to detect relapses and glucocorticoid-related adverse effects. 1, 2, 3

• Monitor for recurrence of headache, scalp tenderness, jaw claudication, and visual symptoms 9
• Track ESR and CRP regularly to guide treatment decisions 2, 3, 9

Critical Pitfalls to Avoid

• Never delay treatment while awaiting biopsy results—vision loss is often irreversible and occurs in 15-35% of patients, primarily before treatment initiation 2, 3
• Do not use alternate-day glucocorticoid therapy—it results in significantly lower remission rates and cyclic symptoms 1, 4
• Avoid rapid steroid withdrawal—this leads to disease exacerbation 3
• Do not use infliximab—it is associated with recurrent ocular symptoms and disease activity 3, 8
• Without treatment, the risk of vision loss in the second eye approaches 50% if one eye is already affected 2, 3