Recurrent Temporal Arteritis with Previous Negative Biopsy
This patient should be immediately restarted on high-dose prednisone 40-60 mg daily for presumed giant cell arteritis (temporal arteritis), given the classic presentation of unilateral temporal headache with pain radiating behind the eye and previous response to corticosteroids, despite the negative biopsy. 1
Clinical Reasoning
Why This is Likely Giant Cell Arteritis (GCA)
- Classic presentation: Unilateral temporal headache with pain radiating behind the eye in a patient over 50 years old is pathognomonic for GCA 1
- Previous corticosteroid response: The fact that prednisone completely resolved identical symptoms last year strongly supports an inflammatory/autoimmune etiology 1
- Negative biopsy does not exclude GCA: Temporal artery biopsies have skip lesions and can be falsely negative in 10-40% of cases, particularly if the patient received even brief corticosteroid treatment before biopsy 1
Immediate Treatment Protocol
Start prednisone 40-60 mg daily immediately - do not wait for additional testing, as vision loss from GCA can occur rapidly and is often irreversible 1
- The initial dose should be maintained for 2-4 weeks until symptoms completely resolve and inflammatory markers normalize 1
- After symptom control, begin gradual taper by 5 mg weekly until reaching 10 mg/day, then taper by 2.5 mg every 2-4 weeks 1
- Total treatment duration is typically 12-24 months 1
Essential Workup (But Don't Delay Treatment)
Obtain these tests immediately, but start prednisone first:
- ESR and CRP - typically markedly elevated in GCA (ESR often >50 mm/hr, frequently >100 mm/hr) 1
- Complete blood count - may show normocytic anemia and thrombocytosis 1
- Comprehensive metabolic panel - baseline before long-term corticosteroids 1
Consider temporal artery ultrasound or MRI if available, as these can show the characteristic "halo sign" of vessel wall inflammation even after biopsy 1
Critical Pitfalls to Avoid
Vision Loss is the Emergency
- Permanent vision loss occurs in 15-20% of untreated GCA patients and can happen suddenly 1
- Once vision loss occurs, it is typically irreversible even with treatment 1
- Any visual symptoms (blurred vision, diplopia, amaurosis fugax) require immediate high-dose IV methylprednisolone 500-1000 mg daily for 3 days before transitioning to oral therapy 1
Don't Be Falsely Reassured by Negative Biopsy
- The previous negative biopsy should not prevent treatment in this clinical scenario 1
- Biopsy sensitivity is only 60-90% due to skip lesions 1
- Clinical diagnosis with elevated inflammatory markers is sufficient to initiate and maintain treatment 1
Monitoring During Treatment
Assess for corticosteroid-related complications:
- Baseline and annual bone mineral density testing for osteoporosis prevention 1
- Monitor blood pressure, glucose, and weight at each visit 1
- Consider PCP prophylaxis if prednisone >20 mg/day for >12 weeks 2
- Calcium and vitamin D supplementation should be started immediately 1
Monitor disease activity:
- ESR/CRP every 4-6 weeks during taper 1
- Clinical symptoms are more reliable than inflammatory markers for detecting relapse 1
- Relapse rate is 30-50% during the first year of treatment 1
Alternative Diagnosis Consideration
While the presentation is classic for GCA, if symptoms do not respond to prednisone within 48-72 hours, consider:
- Cluster headache - but typically shorter duration episodes (15-180 minutes), multiple attacks per day, and associated with autonomic symptoms
- Trigeminal neuralgia - but pain is typically sharp/electric rather than constant aching
- Intracranial pathology - would require MRI brain if atypical features present
However, given the previous identical presentation that resolved with prednisone, GCA remains the overwhelming most likely diagnosis requiring immediate treatment 1.