What are the symptoms and treatment options for temporal arteritis?

Symptoms and Treatment of Temporal Arteritis

Temporal arteritis (giant cell arteritis) presents with new-onset persistent localized headache (often in the temporal area), jaw claudication, scalp tenderness, and constitutional symptoms, requiring immediate high-dose glucocorticoid therapy (40-60 mg/day prednisone-equivalent) to prevent permanent vision loss.

Key Symptoms and Clinical Findings

Cranial Symptoms

• New-onset persistent localized headache, often in the temporal area 1
• Scalp tenderness 1
• Jaw and/or tongue claudication (highly specific with positive LR of 4.90) 1
• Temporal artery tenderness and/or thickening with or without reduced pulsation (positive LR of 3.14-4.70) 1

Visual Symptoms

• Acute visual symptoms including amaurosis fugax (transient vision loss), acute permanent visual loss, and diplopia 1
• Anterior ischemic optic neuropathy, oculomotor cranial nerve palsy, central retinal artery occlusion 1
• Visual loss is an ophthalmological emergency requiring immediate treatment 2

Systemic Manifestations

• Constitutional symptoms (weight loss >2 kg, low-grade fever, fatigue, night sweats) 1
• Symptoms of polymyalgia rheumatica (shoulder and hip girdle pain and stiffness) 1
• Limb claudication (highly specific with positive LR of 6.01) 1
• Anemia 3

Physical Examination Findings

• Tenderness and/or thickening of the superficial temporal arteries 1
• Reduced pulses/blood pressure of the upper limbs 1
• Bruits, particularly in the axilla 1

Laboratory Findings

• Elevated erythrocyte sedimentation rate (ESR) and/or C-reactive protein (CRP) in >95% of cases 1
• ESR >100 mm/h has a positive LR of 3.11 1
• Absence of ESR >40 mm/h has a negative LR of 0.18 (strong evidence against GCA) 1
• Platelet count >400 × 10³/μL has a positive LR of 3.75 1
• Absence of CRP ≥2.5 mg/dL has a negative LR of 0.38 (evidence against GCA) 1

Diagnostic Approach

Initial Evaluation

• Patients with suspected GCA should be referred urgently for diagnostic work-up 1
• Fast-track referral within 24 hours can reduce the rate of permanent visual impairment 1
• Patients with acute visual symptoms should be seen immediately by an ophthalmologist 1

Confirmatory Testing

• Temporal artery biopsy (TAB) with specimen length >1 cm is recommended 1
• Biopsy should be obtained within 2 weeks of starting glucocorticoids 1
• Initial unilateral biopsy is recommended over bilateral biopsies 1
• Vascular imaging (ultrasound, MRI, PET, CT) can identify large vessel involvement 1
• Diagnosis should be confirmed either histologically or by imaging 1

Treatment Approach

Initial Treatment

• High-dose glucocorticoid therapy (40–60 mg/day prednisone-equivalent) should be initiated immediately 1
• Patients with acute visual disturbance should be treated before confirmatory testing 1
• Delaying glucocorticoid therapy when visual loss is present is the strongest risk factor for permanent blindness 1

Maintenance and Tapering

• Once disease is controlled, taper glucocorticoid dose to 15–20 mg/day within 2–3 months 1
• After 1 year, target dose should be ≤5 mg/day 1
• Flare (relapse) occurs in 34%–62% of patients with glucocorticoid monotherapy 1

Glucocorticoid-Sparing Agents

• Tocilizumab has demonstrated efficacy in reducing glucocorticoid requirements and flare rates 1
• Methotrexate may achieve a modest reduction of cumulative glucocorticoid dose 1
• Other agents under investigation include IL-1beta inhibitors (canakinumab), T-cell costimulation blockers (abatacept), and JAK 1/2 inhibitors (baricitinib) 1

Important Clinical Considerations

Diagnostic Pitfalls

• GCA can mimic other conditions including angle-closure glaucoma, hypertension, migraine, trigeminal neuralgia, and carotid artery disease 4
• No single feature is strong enough to confirm or refute the diagnosis if taken alone 1
• Age <70 years makes GCA less likely (negative LR of 0.48) 1
• Only 15%–20% of patients achieve sustained remission with glucocorticoids alone 1

Monitoring

• Regular assessment for clinical signs and symptoms of active disease 1
• Monitor inflammation markers (ESR, CRP) 1
• Assess for glucocorticoid-related adverse effects 1
• Evaluate for large-vessel complications including aneurysms and vascular stenoses 1

By recognizing the symptoms of temporal arteritis and initiating prompt treatment, permanent vision loss and other serious complications can be prevented in this potentially devastating condition.