What is the recommended initial treatment for a patient over 50 years old presenting with new temporal headache, scalp tenderness, jaw claudication and visual disturbances suggestive of giant cell arteritis?

Initial Treatment for Giant Cell Arteritis

Start high-dose oral prednisone 40–60 mg daily (or 1 mg/kg/day, maximum 60 mg) immediately upon clinical suspicion—do not wait for temporal artery biopsy confirmation—and strongly consider adding tocilizumab 162 mg subcutaneously weekly at diagnosis to reduce glucocorticoid exposure and prevent relapse. 1, 2, 3

Immediate Management Algorithm

For Patients WITHOUT Visual Symptoms or Threatened Vision Loss

• Initiate oral prednisone 40–60 mg daily as a single morning dose the moment GCA is clinically suspected based on new temporal headache, scalp tenderness, jaw claudication, elevated ESR/CRP, and age >50 years 1, 2
• Do not delay treatment while awaiting temporal artery biopsy; irreversible vision loss can occur within hours to days if untreated 1, 2, 4
• Add tocilizumab 162 mg subcutaneously weekly at the time of diagnosis (not reserved for relapse) to achieve glucocorticoid-sparing effects and reduce relapse rates 1, 2, 3

For Patients WITH Visual Symptoms (Amaurosis Fugax, Diplopia, Acute Visual Loss)

• Administer intravenous methylprednisolone 500–1000 mg daily for 3 consecutive days immediately, then transition to oral prednisone 40–60 mg daily 1, 2, 3
• This is the only scenario where IV pulse steroids are indicated; patients with threatened vision loss require this aggressive approach to prevent permanent blindness 1, 2
• Still add tocilizumab after the IV pulse therapy 2, 3

Diagnostic Confirmation (Do Not Delay Treatment)

• Arrange temporal artery biopsy within 2 weeks of starting glucocorticoids; biopsy remains diagnostic even after steroid initiation within this window 1, 2, 4
• Obtain a long-segment biopsy (>1 cm) to improve diagnostic yield because skip lesions can cause false-negative results 1, 2, 4
• If biopsy is negative but clinical suspicion remains high, obtain noninvasive vascular imaging (MR or CT angiography of neck/chest/abdomen/pelvis) to evaluate for large-vessel involvement 2, 3

The 2018 EULAR guidelines emphasize that patients presenting with acute visual disturbance should be treated before the diagnostic appointment, as delaying glucocorticoid therapy when visual loss is present is the strongest risk factor for permanent blindness 1. The 2021 ACR/Vasculitis Foundation guideline conditionally recommends high-dose over moderate-dose glucocorticoids specifically because studies supporting moderate-dose therapy are of low quality 1.

Adjunctive Therapies

Tocilizumab (Preferred Steroid-Sparing Agent)

• Tocilizumab 162 mg subcutaneously weekly (or 8 mg/kg IV every 4 weeks) is FDA-approved for GCA and should be initiated at diagnosis, not reserved for relapse 1, 2, 3, 5
• The 2021 ACR guideline conditionally recommends tocilizumab with glucocorticoids over glucocorticoids alone for newly diagnosed GCA, based on a 2017 trial demonstrating significant glucocorticoid-sparing effects 1
• Tocilizumab reduces relapse rates from 34–75% (with steroids alone) to significantly lower levels 2, 6

Methotrexate (Alternative if Tocilizumab Contraindicated)

• Consider methotrexate 15–25 mg weekly if tocilizumab is unavailable, contraindicated (history of gastrointestinal perforations, diverticulitis, recurrent infections), or cost-prohibitive 1, 2, 3
• Evidence for methotrexate is less robust than tocilizumab, but meta-analyses of three randomized controlled trials support its use to lower recurrence rates and spare steroids 7, 8

Low-Dose Aspirin

• Prescribe aspirin 75–150 mg daily to all GCA patients to reduce cardiovascular and cerebrovascular ischemic events 2, 3

Glucocorticoid Tapering Strategy

• Maintain initial high dose (40–60 mg daily) for approximately 1 month until clinical symptoms resolve and inflammatory markers (ESR/CRP) normalize 1, 2
• Target dose of 15–20 mg daily by 2–3 months, then 10–15 mg daily by 3 months, and ≤5 mg daily by 12 months if disease remains controlled 1, 2
• Taper decisions should be guided by clinical symptoms and ESR/CRP normalization, not by a rigid calendar 1, 2
• Never use alternate-day dosing during taper; the 2021 ACR guideline conditionally recommends daily dosing over alternate-day schedules because it increases relapse risk 1

The EULAR guideline specifies that tapering should not be started until both ESR and CRP have reached their lowest stable levels, which usually takes approximately 2 weeks 1. Total treatment duration typically ranges from 1–2 years, though some patients require longer therapy 2, 6.

Supportive Care and Monitoring

• Initiate bone-protective therapy (calcium, vitamin D, bisphosphonates) for all patients unless contraindicated, as glucocorticoid-related adverse events occur in approximately 86% of patients on long-term therapy 2
• Measure ESR and CRP at each visit to guide treatment decisions and tapering 2, 3
• Obtain baseline vascular imaging (MR or CT angiography) at diagnosis to evaluate large-vessel involvement; periodic imaging may be necessary for patients with known large-vessel disease to assess for aneurysms and stenoses 2, 3

Management of Disease Relapse

• Major relapse (cranial ischemic symptoms): Increase prednisone to 40–60 mg daily immediately and add tocilizumab (preferred over methotrexate for relapsing disease with cranial symptoms) 2
• Minor relapse (constitutional symptoms only): Modestly increase glucocorticoid dose, slow the taper, and consider initiating or optimizing a steroid-sparing agent 2

Critical Pitfalls to Avoid

• Never delay high-dose glucocorticoid treatment while awaiting biopsy confirmation or specialist consultation; untreated GCA carries a 14–50% risk of permanent vision loss 1, 2, 4
• Do not rely solely on ESR/CRP for treatment decisions; clinical assessment should take precedence over isolated laboratory elevations 1, 2
• Do not use infliximab for GCA, as it is linked to recurrent ocular symptoms and persistent disease activity 2
• Do not obtain biopsy specimens shorter than 1 cm, as skip lesions can lead to false-negative results 1, 2, 4

The evidence strongly supports immediate empiric treatment with high-dose glucocorticoids for all patients with suspected GCA, even before diagnostic confirmation. The addition of tocilizumab at diagnosis represents a paradigm shift from older guidelines that reserved steroid-sparing agents for relapsing disease; current evidence demonstrates superior outcomes when tocilizumab is initiated early rather than waiting for treatment failure 1, 2, 3.