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Giant cell arteritis with normal erythrocyte sedimentation rate: a management dilemma
1Department of Ophthalmology of the Medical College of Pennsylvania.
Insights
Cranial arteritis can present with normal erythrocyte sedimentation rates, leading to delayed diagnosis and potential vision loss. Prompt steroid treatment is crucial, even before biopsy confirmation, in suspected cases of giant cell arteritis.
Area of Science:
- Rheumatology
- Ophthalmology
- Neurology
Background:
- Giant cell arteritis (GCA), also known as cranial arteritis, is a systemic vasculitis affecting medium to large arteries.
- Diagnosis typically relies on elevated erythrocyte sedimentation rate (ESR) and temporal artery biopsy (TAB).
- However, atypical presentations with normal ESR can occur, posing diagnostic challenges.
Observation:
- This report details two cases of cranial arteritis in patients with normal ESR (13 mm/hr and 26 mm/hr).
- One patient experienced irreversible blindness due to diagnostic delay while awaiting TAB.
- The second patient presented complex management challenges balancing steroid efficacy against adverse effects.
Findings:
- Normal ESR does not exclude active cranial arteritis.
- Clinical suspicion and prompt initiation of treatment are critical for preserving vision.
- Diagnostic delays can lead to severe, irreversible complications.
Implications:
- Physicians must maintain a high index of suspicion for GCA, even with normal inflammatory markers.
- Empirical steroid treatment should be initiated immediately upon suspicion, concurrent with ESR testing, to prevent vision loss.
- Temporal artery biopsy remains important but should not delay urgent corticosteroid therapy in suspected GCA cases.
Abstract:
Two patients with cranial arteritis and normal erythrocyte sedimentation rates of 13 mm/hr. and 26 mm/hr. are presented. One patient became totally blind awaiting temporal artery biopsy. The other patient was a challenge in titrating increased steroid dose with cardiovascular complications against decreased steroid dose with worsening of visual and neurologic problems. Available tests may be negative in active disease. The clinical picture is paramount in decision making, and a high index of suspicion is mandatory. Since a delay of twenty-four hours may be critical, steroids usually should be started as soon as an erythrocyte sedimentation rate has been drawn, without waiting for a biopsy. Once steroid therapy has been started, temporal artery biopsy should be performed conveniently soon, regardless of the erythrocyte sedimentation rate.