This case demonstrates BCCAO in a patient with primary Sjogren’s syndrome, HTN, and DM. The most common risk factors of CCAO include HTN and a history of smoking [6]. Previous studies also reported that DM is a major risk factor for atherosclerosis which causes carotid artery occlusion [7]. Though this patient denied a smoking history, the possibility of BCCAO due to HTN and DM, in this case, cannot be fully ruled out. However, in patients with HTN and DM, the progression of atherosclerosis should be detectable in MRI [10]. Eccentric wall thickening and the juxtaluminal T2-weighted hyperintensity that is evident in atherosclerotic disease were not seen in our patient. Another reason for HTN and DM may be the minor cause of CCAO in this case is that this patient was a 50-year-old female, both the age and the gender were possibly protective against the ischemic stroke [11].
The radiologic finding of MRA in this patient suggested that nonatherosclerotic causes of the large vessel occlusion, such as inflammatory diseases, were more likely. The possibilities considered were Sjögren’s syndrome and Takayasu arteritis. The age at disease onset, the absence of narrowing of the aorta and/or its primary branches in MRI, and the absence of claudication of the extremities suggest Takayasu arteritis is less likely according to the American College of Rheumatology (ACR) classification criteria [12]. Sjögren’s syndrome is a challenging disorder characterized by several clinical features in different systems, including ocular, cutaneous, and vascular domains [13]. The risk of large-artery involvement in Sjögren’s patient is less often discussed [3]. There are chances that the inflammatory changes due to Sjögren’s syndrome can cause vascular occlusion, but current evidence is not sufficient. This case provides further insight into the complex pathogenesis of the carotid artery involvement in Sjogren’s syndrome, HTN, and DM.
As the ophthalmic artery is a branch of the carotid artery, OIS occurs after the stenosis of the carotid artery [8]. On the other hand, Sjögren’s syndrome can cause systemic inflammation, leading to retinal vasculitis which may also cause visual dysfunction [1]. Therefore, we cannot exclude the possibility that the visual dysfunction in the right eye previously diagnosed as retinal vasculitis was also coincidentally caused by OIS due to BCCAO.
CCAO is an uncommon cause for OIS [9]. The most common clinical features of OIS are rubeosis iridis in the anterior segment and narrowed retinal arteries, dilated and not tortuous retinal veins, retinal hemorrhage, and microaneurysms in the posterior segment [14]. Another characteristic of OIS is ocular or periocular pain due to ocular ischemia [14]. There was no anterior segment sign in this case during the first visit, and the patient also denied ocular pain. Ophthalmologists should be aware that sonography of the ophthalmic artery and carotid artery imaging are important in the differential diagnosis for patients with such atypical presentation.
The management of unilateral CCAO is still debatable because of the low incidence of CCAO [15]. There are several surgical approaches for patients with unilateral CCAO, however, complications from surgery include periprocedural stroke, periprocedural mortality, ipsilateral stroke, restenosis, and re-occlusion [15]. Most patients with unilateral CCAO (68%) received medical treatment instead of surgery [16]. For patients with BCCAO, it is still unknown whether medical or surgical management is optimal due to the limited sample size of previous studies [16].
In summary, we presented a Sjögren’s syndrome patient with OIS due to BCCAO. Large vessel abnormalities should be considered when acute visual loss is found in a patient with Sjögren’s syndrome, HTN, or DM. Various imaging modalities including brain MRA, ultrasonography of the ophthalmic artery, aortography, and CTA are useful for diagnosing diseases with complicated pathogenesis, such as in this case.
