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Background  Sjögren’s syndrome may be a risk factor for carotid artery stenosis. Bilateral common carotid artery occlusion (BCCAO) in a patient with Sjögren’s syndrome was not reported before. In this report, we describe a female with Sjögren’s syndrome who had acute visual loss due to ocular ischemic syndrome (OIS) with BCCAO.    Case presentation  A 50-year-old female with Sjögren’s syndrome visited our clinic with acute visual loss in the left eye. The best corrected visual acuity (BCVA) was 2/100 in the left eye, and the intraocular pressure (IOP) was normal in both eyes. Ocular ischemic change was observed during the ophthalmic examination. Aortography and computed tomography angiography (CTA) showed nearly total occlusion of the bilateral CCA. Thus, OIS with BCCAO was diagnosed. The vision in the left eye improved to 30/100 after carotid artery stenting for the left common carotid artery.    Conclusions  BCCAO may be present in patients with Sjögren’s syndrome. Large vessel abnormalities should be considered when acute visual loss is found in a patient with Sjögren’s syndrome.

Acute visual loss as the first ocular symptom in a Sjögren’s syndrome patient with bilateral common carotid artery occlusion: a case report