When the picture masks the diagnosis - an atypical and severe giant cell arteritis case report.

ABSTRACT

PURPOSE: To describe an atypical and severe case of Giant Cell Arteritis (GCA). METHODS: Case report description. RESULTS: We report the case of a 76-year-old male who presented with severe and progressive bilateral visual loss. Upon presentation at the ophthalmology emergency room, the patient's right eye (OD) displayed aqueous flare, hyphema, rubeosis iridis, and dense posterior capsular opacification. After YAG laser capsulotomy, vitreous densifications, intraretinal hemorrhages, cotton wool spots, retinal ischemia and vasculitis were observed in the fundoscopy. The patient's left eye (OS) also presented intraretinal hemorrhages and cotton wool spots around the temporal arcades. The diagnostic workup excluded infectious diseases, demyelinating diseases, and ocular ischemic syndrome due to carotid obstruction. Proteinogram revealed a monoclonal gammopathy, suggesting a possible hematologic condition. High-dose corticotherapy was initiated, which improved the vitreous densifications and enabled the visualization of a OD pale optic disc. The remaining study did not confirm the diagnosis of hematologic disease. During follow-up, bilateral visual acuity deteriorated, with development of progressive pallor in the OS optic disc. Follow-up fluorescein angiography demonstrated progressive retinal and choroidal ischemia. Finally, due to high clinical suspicion, temporal artery doppler ultrasound was performed, confirming the diagnosis of GCA. CONCLUSION: GCA may present multiple ocular features. The knowledge of these different presentations, including retinal and choroidal ischemia or uveitis, is critical for timely diagnosis and treatment initiation. Since patients with GCA often present with vision loss, ophthalmologists may be the first medical doctors that contact with these patients, being on the frontline of GCA diagnosis.