Sudden visual loss in a 74-year-old with jaw claudication: steroids, biopsy timing and the second eye
A 74-year-old woman presents to the emergency department with sudden painless loss of vision in the left eye on waking 36 hours earlier. Acuity is counting fingers OS and 6/7.5 OD. There is a left relative afferent pupillary defect, a swollen pale optic disc with two flame haemorrhages at the margin, and no vitritis. She mentions intermittent jaw pain when chewing for the past three weeks, scalp tenderness when brushing her hair, and 4 kg of unintentional weight loss. Erythrocyte sedimentation rate is 88 mm/h and C-reactive protein 52 mg/L. Haemoglobin is 108 g/L and platelets are 480 × 10⁹/L. She takes ramipril and atorvastatin and has no known drug allergy. The on-call medical team has already given a single 60 mg dose of oral prednisolone and asks you to review her before she goes to the ward.
1. What is the diagnosis, and how should her corticosteroid treatment be changed now? Justify the dose and route.
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The picture is giant cell arteritis with arteritic anterior ischaemic optic neuropathy: age over 50, sudden painless loss with an altitudinal or diffuse field defect, a swollen pale disc with splinter haemorrhages, a relative afferent pupillary defect, and systemic features - jaw claudication, scalp tenderness, weight loss - with ESR 88 mm/h, CRP 52 mg/L, anaemia and thrombocytosis.
A single 60 mg oral dose is inadequate for sight-threatening disease. The fellow eye is at substantial risk of involvement within days to weeks if untreated or under-treated, and that eye is the only remaining hope for useful vision. She needs high-dose intravenous methylprednisolone, typically 500–1000 mg daily for three days, followed by oral prednisolone 1 mg/kg/day (usually 60 mg, and up to 80 mg in some protocols). Intravenous pulses are preferred when there is established visual loss with a threatened fellow eye, because they achieve higher tissue concentrations faster and have been associated with a better chance of recovering some vision in the affected eye and protecting the other.
Before and during the pulses, check blood glucose, blood pressure, renal function, electrolytes and baseline bone protection; arrange calcium and vitamin D with a bisphosphonate, a proton pump inhibitor, and consider low-dose aspirin. Do not delay treatment for biopsy, imaging or a rheumatology opinion - treatment precedes confirmation when the clinical suspicion is this high.
2. How and when should temporal artery biopsy be arranged, and what would you do with a negative result?
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Arrange temporal artery biopsy within 1–2 weeks of starting glucocorticoids. Steroids do not reliably abolish the histological picture in that window, but delays beyond two weeks increase the chance of a false negative as the inflammatory infiltrate resolves. A positive biopsy confirms the diagnosis but does not change acute management, which must never wait on it.
Technique matters: a segment at least 1–2 cm long should be excised, because the disease is segmental (skip lesions) and short specimens produce false negatives. Some clinicians take the contralateral artery if the first is negative but suspicion remains high; a unilateral biopsy is sufficient initially. Temporal artery ultrasound showing a hypoechoic halo, or MRI of the superficial temporal arteries, are non-invasive alternatives where expertise exists, but neither replaces biopsy in a patient already committed to long-term steroids.
A negative biopsy does not exclude giant cell arteritis. Up to a fifth of clinically typical cases are biopsy-negative, and the diagnosis remains clinical. If the history, examination and inflammatory markers are compelling, continue glucocorticoids and treat to a clinical endpoint; consider a contralateral biopsy or an alternative diagnosis (non-arteritic ischaemic optic neuropathy, but the systemic features here are against it) rather than stopping treatment to prove a point.
3. She responds well and asks how long she will need steroids. Outline the taper, monitoring, relapse features and the effect on her other vascular risk.
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Treat with prednisolone 1 mg/kg/day for the first 2–4 weeks, then taper slowly - for example by 5 mg every 2 weeks to 20 mg, then by 2.5 mg every 2–4 weeks - guided by symptoms, ESR and CRP, but not by markers alone; markers can be falsely reassuring or persistently abnormal for other reasons. Most patients need 1–2 years of treatment, and a substantial minority relapse during tapering.
Relapse features to warn her about: return of headache, jaw claudication, scalp tenderness, polymyalgic limb girdle stiffness, transient visual obscurations, or any new visual loss in either eye - the last requires urgent review and a return to high-dose steroid. A rising ESR or CRP alone, without symptoms, warrants review rather than an automatic dose increase.
Monitoring and prevention during the taper: bone density and fracture risk (bisphosphonate, calcium and vitamin D), blood pressure, HbA1c and lipids, gastric protection, and cataract and glaucoma surveillance. Low-dose aspirin is often added for its antiplatelet effect, and her statin should continue. If relapses are frequent or the steroid burden is intolerable, a steroid-sparing agent such as tocilizumab or methotrexate can be considered with rheumatology input.
Finally, be honest about prognosis: vision lost at presentation rarely recovers, and the main goal of treatment is protecting the fellow eye and preventing large-vessel complications such as aortic aneurysm and stenosis, which require long-term surveillance.