Immediate High-Dose Glucocorticoid Therapy
This patient requires immediate initiation of high-dose oral prednisone 40-60 mg daily without waiting for diagnostic confirmation, as the clinical presentation of severe jaw pain (jaw claudication), transient vision loss (amaurosis fugax), temporal/scalp tenderness, and elevated ESR is highly suggestive of giant cell arteritis (GCA), which can cause irreversible blindness within days if untreated. 1, 2
Urgent Diagnostic Workup (Same Day or Next Day)
Immediate ophthalmology referral to assess for visual symptoms or subclinical involvement, as patients with visual disturbance should be seen as soon as possible to exclude other causes and evaluate for anterior ischemic optic neuropathy, central retinal artery occlusion, or choroidal ischemia 1, 2
Temporal artery biopsy should be arranged urgently (ideally within 1-2 weeks), but treatment must not be delayed while awaiting biopsy, as histologic changes persist for weeks after glucocorticoid initiation 1, 2
Vascular imaging (ultrasound of temporal arteries, or MRI/CT/PET-CT for aorta and extracranial arteries) to confirm diagnosis and document extent of arterial involvement 1, 2
Clinical Reasoning
The combination of jaw claudication (93% specific for GCA), transient vision blackout (amaurosis fugax), temporal/scalp tenderness, and elevated ESR creates a high pretest probability for GCA. 1, 2 ESR >40 mm/h has 93.2% sensitivity for GCA with a negative likelihood ratio of 0.18. 2, 3 The transient vision loss represents a critical warning sign—permanent visual loss can occur within days of symptom onset if treatment is not initiated immediately. 1
Key pitfall: Do not wait for biopsy results or imaging before starting treatment. 1 Delaying glucocorticoid therapy when visual symptoms are present is the strongest risk factor for permanent blindness. 1 While diagnostic test sensitivity decreases following treatment, imaging and biopsy may show features of GCA even weeks after glucocorticoid therapy. 1
Additional Baseline Assessment
C-reactive protein (CRP) measurement, as it often correlates with ESR and helps confirm systemic inflammation 1, 2
Complete blood count to assess for anemia (common in GCA) 1, 2
Comprehensive metabolic panel including liver function tests and creatinine 2
Check peripheral pulses and blood pressure in both arms to evaluate for large vessel involvement (bruits particularly in the axilla, reduced pulses/blood pressure of upper limbs) 1, 2
Assess for polymyalgia rheumatica symptoms (bilateral shoulder and hip girdle pain with morning stiffness >45 minutes), present in up to 50% of GCA patients 1
Treatment Monitoring Strategy
ESR and CRP should be monitored every 2-4 weeks initially until normalization, then every 6 months during maintenance therapy 2, 3
Gradual glucocorticoid taper after disease control: target dose of 15-20 mg/day within 2-3 months, and after 1 year to ≤5 mg/day 1
Consider glucocorticoid-sparing agents such as tocilizumab or methotrexate adjunctive to steroids to reduce glucocorticoid exposure and lower risk of recurrent stroke 1, 2
Most patients require 2-3 years of treatment, and tapering should be done gradually while monitoring for symptom recurrence 2
Critical Caveats
Do not be falsely reassured by the bilateral 20/30 visual acuity. The transient vision blackout (amaurosis fugax) is a harbinger of permanent vision loss and indicates critical ischemia. 1 Fast-track referral and immediate treatment have been shown in retrospective cohorts to reduce the rate of permanent visual impairment compared with routine non-urgent referral. 1
Intravenous methylprednisolone (1-2 mg/kg or higher dose bolus) should be considered for patients who present with acute visual symptoms, as it may benefit those presenting early following onset of visual symptoms. 1 However, oral high-dose prednisone 40-60 mg daily is the standard initial approach and should be started immediately. 1, 2