Treatment of Giant Cell Arteritis
Initiate high-dose oral glucocorticoids (prednisone 40-60 mg daily) immediately upon clinical suspicion of giant cell arteritis, and strongly consider adding tocilizumab as adjunctive therapy to reduce glucocorticoid requirements and prevent disease relapse. 1, 2
Immediate Management
Initial Glucocorticoid Therapy
- Start prednisone 40-60 mg daily (or 1 mg/kg/day) as a single morning dose without waiting for biopsy confirmation 1, 2
- Daily dosing is superior to alternate-day regimens, which show significantly lower remission rates and cyclic symptom recurrence 1, 3
- For patients with threatened vision loss, amaurosis fugax, or acute visual symptoms, administer IV methylprednisolone 500-1000 mg daily for 3 consecutive days before transitioning to oral therapy 1
- High-dose oral glucocorticoids are generally sufficient for most patients; IV pulse therapy should be reserved specifically for those at highest risk of irreversible vision loss 1
Adjunctive Immunosuppressive Therapy
- Add tocilizumab 162 mg subcutaneously weekly (or 8 mg/kg IV every 4 weeks) at diagnosis to achieve glucocorticoid-sparing effects 1, 4
- Tocilizumab is FDA-approved for GCA and demonstrated significant glucocorticoid-sparing effects in clinical trials 1, 4
- If tocilizumab is unavailable or contraindicated (recurrent infections, history of gastrointestinal perforations, diverticulitis, or cost concerns), consider methotrexate 15-25 mg weekly as an alternative steroid-sparing agent 1
- Abatacept may be considered if tocilizumab and methotrexate are ineffective 1
Aspirin Therapy
- Prescribe low-dose aspirin 75-150 mg daily to all GCA patients to reduce cardiovascular and cerebrovascular ischemic events 1, 2
- This is particularly important for patients with critical or flow-limiting involvement of vertebral or carotid arteries 1
Glucocorticoid Tapering Strategy
Tapering Protocol
- Maintain initial high dose (40-60 mg daily) for one month if disease is controlled 2
- Begin gradual taper guided by clinical symptoms and inflammatory markers (ESR/CRP) 1, 2
- Target dose of 10-15 mg daily at 3 months 2
- Target dose of ≤5 mg daily at 12 months if disease remains controlled 2
- Total treatment duration typically ranges from 1-2 years, though some patients require longer courses 1, 5
Important Tapering Considerations
- The optimal duration is not well-established and should be guided by clinical manifestations, glucocorticoid toxicity, number of flares, and patient preferences 1
- Continue non-glucocorticoid immunosuppressive agents (tocilizumab or methotrexate) during and after glucocorticoid taper 1
- For patients achieving remission on glucocorticoids for 6-12 months, taper off glucocorticoids completely rather than maintaining long-term low-dose therapy 1
Management of Disease Relapse
Relapse with Cranial Ischemic Symptoms
- Increase glucocorticoid dose AND add or switch non-glucocorticoid immunosuppressive agent (e.g., add tocilizumab if not already on it, or switch from methotrexate to tocilizumab) 1
- Do not simply increase glucocorticoids alone for cranial ischemic relapses 1
Relapse with Polymyalgia Rheumatica Symptoms Only
- May be controlled by increasing glucocorticoid dose alone without necessarily adding additional immunosuppression 1
Relapse on Moderate-to-High Dose Glucocorticoids
- Add a non-glucocorticoid immunosuppressive drug, as this indicates glucocorticoids alone will be insufficient for disease control 1
Special Considerations for Elderly Patients with Comorbidities
Glucocorticoid-Related Adverse Events
- Cumulative glucocorticoid exposure is directly associated with increased risk of adverse events, with each 1 gram increase in cumulative dose significantly increasing the likelihood of complications 6
- Most frequent adverse events include hypertension, eye complications, bone health issues, and glucose intolerance 6
- Initiate bone protection therapy (calcium, vitamin D, bisphosphonates) for all patients unless contraindicated 2
Moderate-Dose Glucocorticoid Option
- Moderate-dose glucocorticoids (30-40 mg daily) may be considered for patients with significant risk of severe glucocorticoid toxicity and low risk of vision loss or life-threatening complications 1
- However, this is a conditional recommendation due to low-quality supporting evidence 1
Infection Risk
- Elderly patients are at higher risk for infections, particularly with combined immunosuppression 4
- Monitor closely for opportunistic infections including toxoplasmosis, which can occur with high-dose glucocorticoid therapy 7
Monitoring Protocol
Clinical Assessment
- Assess clinical symptoms at each visit, including headache, jaw claudication, visual symptoms, and constitutional symptoms 2
- Measure four-extremity blood pressures to detect large vessel involvement 2
- Perform vascular examination for new bruits or pulse deficits 2
Laboratory Monitoring
- Measure ESR and CRP at each visit to guide treatment decisions and tapering 1, 2
- Note that inflammatory markers are elevated in >95% of cases at diagnosis but can be normal in some patients with active disease 2
Imaging Surveillance
- Obtain baseline noninvasive vascular imaging (MR or CT angiography of neck/chest/abdomen/pelvis) at diagnosis to evaluate large vessel involvement 1
- For patients with documented large vessel involvement, perform periodic imaging to assess for aneurysms, stenoses, and stability of existing lesions 1, 2
- Patients without large vessel involvement may not require routine repeated imaging 1
Common Pitfalls to Avoid
- Never delay glucocorticoid initiation while awaiting temporal artery biopsy or imaging confirmation 1, 2
- Do not use alternate-day glucocorticoid dosing, as it results in significantly lower remission rates and cyclic symptom recurrence 1, 3
- Do not rely on inflammatory markers alone for disease activity assessment, as they can be normal in active disease 1, 2
- Avoid glucocorticoid monotherapy when possible, as combination therapy with tocilizumab or methotrexate significantly reduces cumulative glucocorticoid exposure and associated toxicity 1, 6
- Do not routinely use IV pulse glucocorticoids for all patients, as they should be reserved for those with threatened vision loss; routine use increases infection risk without clear benefit in most patients 1
- Do not use statins specifically for GCA treatment, as they provide no clinically significant immunosuppressive effect (though they may be warranted for cardiovascular risk reduction based on other indications) 1