Eyelid Swelling As The Initial Presentation Of Sjögren’S Syndrome: A Case Report

To report a rare case of Sjögren’s syndrome who initially presented with eyelid swelling and developed orbital inflammation and posterior scleritis.

Tertiary referal center, University hospital

A 69-year-old woman presented with a 3-day history of right eyelid swelling, erythema, and foreign body sensation. Best-corrected visual acuity was 20/32 and intraocular pressure was 31 mmHg in the right eye. Slit-lamp examination showed diffuse chemosis, subconjunctival hemorrhage, corneal edema with erosion, and a shallow anterior chamber, which was confirmed by anterior segment optical coherence tomography. Extraocular movement was restricted. Orbital imaging demonstrated posterior scleral thickening, proptosis, and medial rectus muscle infiltration with preseptal and postseptal involvement. Fundus fluorescein angiography revealed peripheral leakage and parafoveal choroidal neovascularization, and optical coherence tomography showed choroidal folding. Laboratory tests showed leukocytosis and elevated C-reactive protein. Orbital cellulitis was initially suspected, and intravenous piperacillin/tazobactam was started. Although right-sided inflammation improved, contralateral proptosis and chemosis developed two weeks later. Further evaluation revealed positive anti-SSA/Ro and antinuclear antibodies, decreased salivary gland uptake, and reduced tear secretion, leading to a diagnosis of Sjögren’s syndrome–associated orbital inflammation. Systemic prednisolone and methotrexate led to marked improvement; however, recurrent scleritis developed three months later and fluctuated over six months despite treatment.

Sjögren’s syndrome may present as bilateral orbital inflammation with secondary angle closure and recurrent scleritis, initially mimicking orbital cellulitis. Prompt systemic evaluation and immunosuppressive treatment are essential for appropriate management.