Giant Cell Arteritis in Ophthalmology –⁠ Current Treatment Options. A Review

Overview

Giant cell arteritis (GCA) is a systemic granulomatous vasculitis of medium and large-sized arteries, which represents an ophthalmic emergency because of the risk of sudden, severe and often irreversible loss of vision. Its most important ocular manifestation is arteritic anterior ischemic optic neuropathy; less frequent manifestations include central retinal artery occlusion, cilioretinal artery occlusion, posterior ischemic optic neuropathy, choroidal ischemia and ocular motor nerve palsies. Giant cell arteritis should be actively considered in every patient over 50 years of age presenting with new-onset ischemic optic neuropathy, amaurosis fugax, diplopia or another cranial ischemic manifestation. The diagnosis is based on a combination of the clinical findings, inflammatory laboratory markers, ultrasonography of the temporal and axillary arteries, temporal artery biopsy when appropriate, and additional imaging modalities. However, in patients with high clinical suspicion, treatment must not be deferred until definitive diagnostic confirmation. Systemic glucocorticoids remain the cornerstone of acute therapy; in patients with ocular ischemic involvement, intravenous methylprednisolone pulses followed by high-dose oral glucocorticoids are usually recommended. New glucocorticoid-sparing treatment options, particularly interleukin-6 receptor blockade with tocilizumab and JAK1 inhibition with upadacitinib, expand the possibilities for long-term disease control and reduction of cumulative steroid exposure. Early identification of the pathology by the ophthalmologist and close interdisciplinary collaboration with rheumatologists, internists, neurologists, infectious disease and imaging specialists are essential to prevent bilateral visual loss and systemic ischemic complications.

Keywords:

glucocorticoids – giant cell arteritis – tocilizumab – visual loss – temporal arteritis – arteritic anterior ischemic optic neuropathy – upadacitinib