What is the recommended long-term treatment for giant cell arteritis?

Long-Term Treatment for Giant Cell Arteritis

For long-term management of giant cell arteritis, initiate high-dose oral glucocorticoids (prednisone 1 mg/kg/day, maximum 60 mg/day) combined with tocilizumab as first-line therapy to minimize steroid exposure and reduce relapse rates, with gradual glucocorticoid tapering over 12-24 months guided by clinical symptoms and inflammatory markers. 1, 2

Initial Glucocorticoid Therapy

Start high-dose oral prednisone immediately (1 mg/kg/day, maximum 60 mg/day) upon clinical suspicion, even before biopsy confirmation, to prevent irreversible vision loss. 1, 2

• Use daily dosing rather than alternate-day schedules, as alternate-day therapy increases relapse risk and achieves lower remission rates. 1
• For patients with threatened vision loss or acute visual symptoms, administer IV pulse methylprednisolone (500-1000 mg/day for 3 days) immediately before transitioning to high-dose oral prednisone. 2, 3
• Maintain the initial high dose for approximately one month to ensure adequate disease control before beginning taper. 1, 2

Glucocorticoid-Sparing Agents: The Critical Decision

Add tocilizumab to glucocorticoids as first-line therapy for most patients, particularly those at high risk of steroid-related complications. 1, 2, 4 This represents the most significant advancement in GCA management, with level 1A evidence demonstrating substantial glucocorticoid-sparing effects and reduced relapse rates. 1

• Methotrexate is the alternative if tocilizumab is contraindicated due to recurrent infections, history of gastrointestinal perforations, diverticulitis, or cost considerations. 1, 5, 6 However, methotrexate has weaker evidence (conflicting results in trials) compared to tocilizumab. 1, 6
• Consider adding immunosuppressive agents particularly for patients with active extracranial large vessel involvement, where combination therapy is superior to glucocorticoids alone. 1, 2

Structured Glucocorticoid Tapering Protocol

Follow this specific tapering schedule once symptoms are controlled and inflammatory markers (ESR, CRP) have normalized:

• Month 1: Maintain initial high dose (40-60 mg/day). 1
• Months 2-3: Taper to 10-15 mg/day. 1, 4
• By 12 months: Aim for ≤5 mg/day. 2
• Total duration: Typically 12-24 months, though some patients require several years of low-dose therapy. 1

Guide tapering by clinical symptoms and inflammatory markers, not by arbitrary schedules. 2, 4 Up to 60% of patients experience relapses during tapering, necessitating individualized adjustments. 4

Management of Disease Relapse

For relapses with cranial ischemic symptoms (headache, jaw claudication, visual symptoms):

• Add a non-glucocorticoid immunosuppressive agent (preferably tocilizumab over methotrexate). 2, 4
• Increase glucocorticoid dose. 2, 4

For relapses with polymyalgia rheumatica symptoms only (shoulder/hip girdle pain, stiffness):

• Increasing glucocorticoid dose alone may be sufficient without adding additional immunosuppression. 2

For isolated elevation of inflammatory markers without clinical symptoms:

• Clinical observation and monitoring without escalating therapy is recommended. 2

Essential Adjunctive Therapies

Prescribe low-dose aspirin (75-150 mg/day) for all patients unless contraindicated, as this decreases cranial ischemic complications and cardiovascular events. 2, 7, 5

Implement bone protection therapy for all patients on long-term glucocorticoids, as 86% of patients experience glucocorticoid-related adverse events at 10-year follow-up. 1, 4

Monitoring Strategy

Monitor with both clinical assessment and laboratory markers:

• Track clinical symptoms (headache, jaw claudication, visual changes, constitutional symptoms). 2, 4
• Measure ESR and CRP regularly to guide treatment decisions. 2, 4
• Obtain baseline noninvasive vascular imaging to evaluate large vessel involvement. 2, 4
• Continue long-term monitoring even in apparent remission, as relapses can occur. 2

Critical Pitfalls to Avoid

Never delay treatment while awaiting biopsy results—vision loss occurs in 15-35% of patients and is usually irreversible, with 50% risk of second eye involvement if one eye is already affected. 2, 4

Avoid rapid glucocorticoid withdrawal, as this leads to disease exacerbation and relapse. 1, 2

Do not use infliximab for GCA, as it is associated with recurrent ocular symptoms and persistent disease activity despite initial promising open-label data. 2, 5

Recognize that some patients cannot completely discontinue glucocorticoids due to recurrent disease or secondary adrenal insufficiency, requiring indefinite low-dose maintenance therapy. 1