Giant Cell Arteritis (GCA) is the #1 Diagnosis and Requires Immediate High-Dose Corticosteroids
This 70-year-old woman with right-sided headache, low-grade fevers, jaw pain, scalp tenderness, and shoulder stiffness has Giant Cell Arteritis until proven otherwise, and treatment must begin immediately—before any diagnostic testing—to prevent irreversible blindness. 1, 2
Top 5 Differential Diagnoses
1. Giant Cell Arteritis (GCA) - PRIMARY DIAGNOSIS
- Classic triad present: temporal headache, jaw claudication, and scalp tenderness in a patient >50 years old 1, 2
- Low-grade fever and shoulder stiffness (polymyalgia rheumatica overlap) strongly support this diagnosis 1, 3
- This is a medical emergency due to risk of permanent bilateral blindness 1, 2
2. Polymyalgia Rheumatica (PMR)
- Shoulder stiffness and constitutional symptoms fit this diagnosis 1, 3
- However, the presence of jaw claudication and scalp tenderness elevates concern to GCA, as 15-20% of PMR patients have concurrent GCA 3
3. Temporomandibular Joint Disorder (TMD)
- Jaw pain could suggest TMD, but jaw claudication (pain with chewing that resolves with rest) is pathognomonic for GCA, not TMD 1
- TMD does not cause fever, scalp tenderness, or shoulder stiffness 1
4. Trigeminal Neuralgia
- Facial pain could suggest this, but trigeminal neuralgia presents with paroxysmal sharp, electric shock-like pain lasting seconds to minutes with refractory periods—not continuous aching pain 1, 4
- No fever or systemic symptoms occur with trigeminal neuralgia 1
5. Intracranial Mass or Stroke
- New headache in elderly patient warrants consideration 1
- However, absence of focal neurological deficits, facial droop, or weakness makes this less likely 1
Diagnostic Workup
IMMEDIATE Laboratory Tests (Draw Before Starting Treatment)
- Erythrocyte Sedimentation Rate (ESR) - typically >50 mm/hr in GCA 1, 2
- C-Reactive Protein (CRP) - markedly elevated in GCA 1, 2
- Complete Blood Count (CBC) - to assess for anemia of chronic disease 5, 3
- Comprehensive Metabolic Panel (CMP) - baseline before starting corticosteroids 3
Critical Pitfall: Do NOT delay treatment while awaiting these results—draw the labs and immediately start corticosteroids 1, 2
Temporal Artery Biopsy
- Gold standard for diagnosis but can be performed within 2 weeks after starting corticosteroids without affecting diagnostic yield 1, 2
- Arrange urgently but do not delay treatment 1, 2
Imaging
- NOT indicated as first-line when GCA is clinically suspected—neuroimaging wastes precious time 2
- Color duplex ultrasonography of temporal arteries may be helpful if biopsy unavailable, but clinical diagnosis takes precedence 3
- MRI or CT only if alternative diagnoses need exclusion after GCA treatment initiated 1
Management of Giant Cell Arteritis (#1 Diagnosis)
Pharmacotherapy - IMMEDIATE INITIATION
High-Dose Corticosteroids (Start Immediately)
- Prednisone 40-60 mg daily orally if no visual symptoms 1, 2
- Methylprednisolone 1000 mg IV daily for 3 days if any visual disturbances present, then transition to oral prednisone 1, 2
- Treatment duration: typically 1-2 years with gradual taper beginning after 2-3 months 1, 3
- Do not wait for ESR/CRP results or biopsy confirmation 1, 2
Adjunctive Therapy
- Tocilizumab (IL-6 inhibitor) - FDA-approved for GCA, consider adding to initial corticosteroid therapy to reduce relapse rates and cumulative steroid exposure 3
- Methotrexate - may be considered as steroid-sparing agent in high-risk relapse patients 1, 3
- Low-dose aspirin 81 mg daily - consider for cardiovascular protection, though evidence is mixed 3
Monitoring During Treatment
- ESR and CRP every 2-4 weeks initially, then monthly during taper 3
- Monitor for corticosteroid adverse effects: blood glucose, blood pressure, bone density, ophthalmologic exams 3
- Approximately 50% of patients relapse during steroid taper, requiring dose adjustment 1, 3
Non-Pharmacotherapy
Immediate Consultations
- Neuro-ophthalmology or ophthalmology - urgent referral to facilitate temporal artery biopsy and monitor for visual complications 1, 2
- Rheumatology - for long-term management and steroid-sparing strategies 3
Supportive Measures
- Calcium 1200-1500 mg daily and Vitamin D 800-1000 IU daily for bone protection during prolonged corticosteroid therapy 3
- Proton pump inhibitor if high risk for peptic ulcer disease 3
- Physical therapy for shoulder stiffness from polymyalgia rheumatica component 6, 3
Red Flags Requiring Immediate Action
Vision-Threatening Symptoms (Initiate IV Corticosteroids)
- Visual disturbances, blurred vision, diplopia, or amaurosis fugax 1, 2
- These indicate impending anterior ischemic optic neuropathy—permanent blindness can occur within days 1, 2, 7
Other Urgent Red Flags
- Sudden severe headache or change in headache pattern 1
- Tongue pain or cyanosis (lingual artery involvement) 1
- Stroke symptoms or focal neurological deficits 1
Common Pitfall: Never attribute new headache in elderly patients to migraine or tension headache without first excluding GCA 2
Return Precautions for Patient
Instruct patient to return immediately or call 911 if:
- Any vision changes, including blurriness, double vision, or vision loss 1, 2
- Sudden severe headache or worsening headache despite treatment 1
- New weakness, numbness, or difficulty speaking 1
- Jaw pain that worsens or tongue pain develops 1
Routine follow-up:
- Return in 2-3 days to assess treatment response and review biopsy results 3
- Weekly visits initially, then every 2-4 weeks during steroid taper 3
- Report any new symptoms immediately, as approximately 50% experience relapse during taper 1, 3
Patient Disposition
Admit to hospital if:
- Any visual symptoms present or threatened 1, 2
- Severe systemic symptoms or inability to take oral medications 3
- Need for IV corticosteroid therapy 1, 2
Discharge home if:
- No visual symptoms 3
- Able to take oral prednisone 3
- Reliable for close follow-up 3
- Ensure temporal artery biopsy scheduled within 1-2 weeks 1, 2
- Ensure ophthalmology/neuro-ophthalmology follow-up within 24-48 hours 1, 2
Critical Point: The priority is preventing permanent bilateral blindness through immediate corticosteroid therapy—this single intervention dramatically reduces morbidity and preserves quality of life 1, 2