Bilateral amaurosis in 11 patients with giant cell arteritis confirmed by arterial biopsy

Background: Even in the times of corticosteroids giant cell arteritis may l ead to complete bilateral blindness. Aim: To assess the frequency of comple te irreversible blindness in giant cell arteritis. Patients and methods: Among all 218 patients with the diagnosis of giant ce ll arteritis confirmed by arterial biopsy between 1980 and 2000, clinical d ata of patients with bilateral amaurosis were further investigated. The mai n interest was focussed on the kind of ocular manifestation, the interval b etween first symptoms and therapy and the interval between involvement of t he first and second eye. Results: In 11 patients (9 women, 2 men, mean age: 79 years) giant cell art eritis led to complete bilateral blindness. Morphological ocular changes we re anterior ischemic optic neuropathy (15 eyes), optic atrophy (4 eyes), po sterior ischemic optic neuropathy (2 eyes), and central artery occlusion (1 eye). The median interval between involvement of the first eye and initiat ion of therapy was 4 days (1/2 day to 8 weeks). The median interval between visual loss in the first and second eye measured 4 days (simultaneously to 30 days). In 2 patients visual loss occurred 1 and 2 days after initiation of treatment (500 mg methylprednisolone/daily), respectively. Treatment wi th corticosteroids (100-1000 mg) did not result in visual improvement in an y patient. Conclusions: Complete bilateral blindness occurred in 5% of patients with g iant cell arteritis, up to 2 days after initiation of treatment with cortic osteroids. This number can only be further reduced by immediate therapy aft er clinical suspicion of giant cell arteritis.