What are the typical presentation and treatment of temporal arteritis?

Temporal Arteritis: Clinical Presentation and Treatment

Immediate Recognition is Critical

Temporal arteritis (giant cell arteritis) requires immediate high-dose glucocorticoid therapy to prevent permanent vision loss, and should be suspected in any patient over 50 years old presenting with new-onset persistent headache, jaw claudication, or visual symptoms. 1

Clinical Presentation

Highly Specific Symptoms (Rule-In Disease)

• Jaw or tongue claudication (pain with chewing/speaking) is the most diagnostically valuable symptom with a positive likelihood ratio of 4.90 1, 2
• Temporal artery abnormalities including tenderness, thickening, or reduced pulsation have positive likelihood ratios of 3.14-4.70 2, 1
• These findings should prompt immediate treatment initiation even before confirmatory testing 1

Common but Less Specific Symptoms

• New-onset persistent headache (often temporal/unilateral) occurs in two-thirds of patients, though it has limited diagnostic specificity 2
• Scalp tenderness is common but not diagnostically discriminating 2, 3
• Constitutional symptoms including weight loss, night sweats, malaise, and fever occur in 50% of cases 2
• Visual symptoms including diplopia, amaurosis fugax, or blurriness occur in 20% and represent impending vision loss 2, 4

Critical Pitfall

While headache and constitutional symptoms are frequently present, their absence does not exclude the diagnosis—focus on jaw claudication and temporal artery findings as the most reliable clinical indicators 2, 1

Laboratory Findings

Inflammatory Markers

• ESR >50 mm/h has a positive likelihood ratio of 1.40, while ESR >100 mm/h increases this to 3.11 2, 1
• Elevated CRP (≥2.5 mg/dL) has a positive likelihood ratio of 1.73, and its absence has a negative likelihood ratio of 0.38 2, 1
• Platelet count >400 × 10³/μL has a positive likelihood ratio of 3.75 2, 1
• Over 95% of cases show elevated ESR and/or CRP 1

Important Caveat

Normal inflammatory markers do not exclude the diagnosis in patients with highly suggestive clinical features—disease progression can occur despite normal marker levels 2

Diagnostic Approach

Urgent Referral Protocol

• Refer within 24 hours for fast-track diagnostic work-up to reduce permanent visual impairment 1
• Do not delay treatment while awaiting confirmatory testing if clinical suspicion is high 1, 4

Confirmatory Testing

• Temporal artery biopsy with specimen length >1 cm is the gold standard for diagnosis 1
• Ultrasound of temporal arteries showing the "halo sign" has 77% sensitivity and 96% specificity compared to clinical diagnosis 2
• If temporal artery ultrasound is negative but suspicion remains, examine axillary arteries 2
• Vascular imaging (MRI, PET, CT) can identify large vessel involvement but is not recommended for cranial artery assessment due to lack of evidence and radiation exposure 2, 1

Treatment Algorithm

Immediate Initiation (Day 1)

Start high-dose glucocorticoids immediately upon clinical suspicion—do not wait for biopsy results 1, 2

• Prednisone 40-60 mg/day (or equivalent) is the standard initial dose 2, 1
• Recent evidence suggests 30-40 mg/day may have similar efficacy 2
• This prevents the most devastating complication: permanent bilateral blindness from anterior ischemic optic neuropathy 5, 4

Tapering Schedule (Months 1-12+)

• Months 2-3: Taper to 15-20 mg/day once disease is controlled 1
• Month 12: Target dose ≤5 mg/day 1
• Total duration: Typically 1-2 years to prevent recurrence 2, 1
• Relapse rate: Nearly 50% of patients relapse during tapering 2

Steroid-Sparing Agents

• Tocilizumab has demonstrated efficacy in reducing glucocorticoid requirements and flare rates 1
• Second-line immunosuppressive agents (methotrexate, azathioprine) have been used in Takayasu arteritis but do not appear to modulate disease progression in giant cell arteritis 2

Monitoring Strategy

Regular Assessment Should Include

• Clinical evaluation for signs and symptoms of active disease 1
• Inflammatory markers (ESR, CRP) monitoring, though these are imperfect indicators of disease activity 2, 1
• Glucocorticoid-related adverse effects screening 1
• Vascular imaging (ultrasound, MRA, CTA) for long-term monitoring of structural damage including stenosis, occlusion, dilatation, or aneurysms—frequency determined individually 2

Flare Management

Imaging may help confirm or exclude suspected flares, but is not routinely recommended for patients in clinical and biochemical remission 2