What is the diagnosis and treatment for a patient over 50 with headache, neck pain, and temporal disc pallor?

Giant Cell Arteritis (GCA) with Optic Nerve Ischemia

This presentation of headache, neck pain, and temporal disc pallor in a patient over 50 is giant cell arteritis with optic nerve ischemia until proven otherwise, and you must initiate high-dose corticosteroids (prednisone 40-60 mg/day) immediately—before any diagnostic testing—to prevent irreversible bilateral blindness. 1, 2, 3

Immediate Management Priority

Start prednisone 40-60 mg/day right now, before drawing labs or ordering imaging. 4, 2, 3 The temporal disc pallor indicates optic nerve ischemia has already occurred, meaning the window to prevent contralateral eye involvement is measured in hours to days, not weeks. 2, 3 Untreated GCA carries a 14-50% risk of permanent vision loss, and the fellow eye is at immediate risk. 1, 3

Critical Actions in Order:

1. Initiate prednisone 40-60 mg/day immediately upon clinical suspicion 4, 2, 3
2. Draw ESR and CRP (but do not wait for results to start treatment) 4, 1, 2, 3
3. Arrange temporal artery biopsy within 2 weeks (can be done after starting steroids without affecting diagnostic yield) 1, 2, 3, 5
4. Add aspirin 75-150 mg/day for cardiovascular protection 1

Why This Diagnosis is Certain

The triad you describe is pathognomonic for GCA:

• Age over 50: GCA essentially never occurs before age 50, and your patient meets this criterion 4, 1
• Headache with neck pain: New headache occurs in 67-90% of GCA cases, and neck pain reflects vascular inflammation of cervical structures 4, 1, 2
• Temporal disc pallor: This represents optic nerve ischemia from arteritic anterior ischemic optic neuropathy—the most feared complication of GCA 4, 2, 3, 6

The combination of temporal region pain with bilateral optic nerve pallor mandates urgent GCA workup even if vision is currently normal, because this indicates active ischemic process. 2

Diagnostic Confirmation (After Treatment Started)

Laboratory Testing:

• ESR and CRP are elevated in >95% of GCA cases at diagnosis 4, 1, 3
• ESR >100 mm/h has positive likelihood ratio of 3.11 1
• However, approximately 11% of GCA patients have normal inflammatory markers, so clinical suspicion overrides laboratory values 3
• Do not withhold treatment if ESR/CRP are only mildly elevated or even normal 1, 2, 3

Temporal Artery Biopsy:

• Gold standard for diagnosis, requiring specimen ≥1 cm length 4, 1, 5, 7
• Sensitivity 67-97%; bilateral biopsies increase yield by 5-10% 4
• Can be performed up to 2 weeks after starting corticosteroids without affecting diagnostic yield 3, 5, 7
• Histopathology shows arterial wall thickening, narrowed lumen, mononuclear inflammatory cells with media invasion, and occasionally multinucleated giant cells 4, 1

Alternative Imaging (if biopsy negative but suspicion remains high):

• Vascular ultrasound showing "halo sign" (circumferential wall thickening) 4, 1
• MRI, CT, or PET-FDG showing arterial wall inflammation 4, 1

Additional Clinical Features to Assess

Ask specifically about these highly specific symptoms:

• Jaw claudication (pain with chewing): Most specific symptom with positive likelihood ratio of 4.90, present in ~50% of cases 4, 1, 3
• Scalp tenderness: Positive likelihood ratio of 3.14 1
• Visual disturbances: Transient vision loss (amaurosis fugax) occurs in 20% before permanent blindness 4, 1
• Constitutional symptoms: Low-grade fever, malaise, weight loss, proximal myalgias (polymyalgia rheumatica) 4, 6

Examine the temporal arteries for:

• Thickening (positive likelihood ratio 4.70, specificity 90.6%) 1, 3
• Loss of pulse (positive likelihood ratio 3.25, specificity 88.2%) 1, 3
• Tenderness to palpation 3, 6

Complete Ophthalmic Examination Required

• Fundus examination: Look for optic disc edema (acute) or pallor (chronic ischemia) 2
• Pupillary examination: Check for afferent pupillary defect indicating optic nerve dysfunction 2
• Visual field testing: May reveal defects despite normal acuity 2
• Cranial nerve assessment: Evaluate for diplopia from ischemia of ocular muscles 2, 6

Treatment Duration and Monitoring

• Initial high-dose prednisone 40-60 mg/day continues until symptoms resolve and inflammatory markers normalize (typically 2-4 weeks) 4
• Gradual taper over 12-18 months guided by clinical symptoms and ESR/CRP levels 1, 7
• 34-62% of patients experience flares during taper, requiring dose adjustment 4
• Consider tocilizumab as adjunctive therapy to reduce glucocorticoid requirements and flare rates 4, 1

Critical Pitfalls to Avoid

1. Never delay corticosteroids while awaiting ESR/CRP results or temporal artery biopsy when GCA is suspected with visual symptoms 2, 3
2. Do not attribute symptoms to migraine in elderly patients with new-onset headache after age 50, especially with jaw claudication 3
3. Do not pursue neuroimaging first when GCA is suspected based on classic symptoms—this wastes precious time 3
4. Do not stop treatment based on negative biopsy alone if clinical suspicion remains high; biopsy sensitivity is imperfect and skip lesions occur 3, 7

Why Immediate Treatment Trumps Diagnosis

The priority is preventing permanent bilateral blindness, which occurs in untreated GCA when the contralateral eye becomes involved. 3 Immediate corticosteroid therapy dramatically reduces this risk and represents the single most important intervention to preserve quality of life. 3 Prednisone therapy dramatically diminishes all symptoms of GCA, including resolution of headache and prevention of further vascular complications. 4