Sjögren Syndrome Presenting with Visual Disturbances

Cranial MRI
  • Sjögren syndrome is a progressive and chronic autoimmune disease, predominantly affecting the lacrimal and the salivary glands.
  • It may be primary or in association with other autoimmune diseases, secondary SS is the latter.
  • Visual disturbances due to optic perineuritis may be the initial presentation in some patients.
  • Optic perineuritis is an inflammation of the optic nerve sheath.
  • Nerve damage is visible on Contrast-enhanced magnetic resonance imaging.
  • High dose methylprednisone therapy early on in the disease has shown promising results.

A 68-year-old female presented with progressive, subacute changes in the visual acuity along with retrobulbar pain bilaterally for the past 4 months. Eventually, after the following sequence of workup, doctors diagnosed her with Sjögren syndrome.


The fundoscopic examination was normal. The direct and consensual motor light reflex was reduced.

Schirmer test was performed, and the patient was diagnosed with Sicca syndrome.

Lab investigations for Sjögren Syndrome:

Serological investigations revealed a positive Rheumatoid factor (value of 23.6) and positive anti-RO/LA antibody.

Imaging to determine the cause of Sjögren Syndrome:

A contrast (gadolinium)-induced Magnetic resonance imaging of the cranium revealed a very thin and intense contrast enhancement (high-intensity thickening) along the optic nerve sheaths bilaterally. MRI also revealed parotid edema. There was no evidence of secondary Sjogren syndrome; therefore, a diagnosis of primary SS was confirmed.

The treating physicians started her on pulse methylprednisone therapy. The patient showed partial improvement in visual acuity and retroocular pain. She was given Rituximab maintenance therapy.


Oikawa JET, de Souza JGL, Ferreira VCC, Wille PR, Parolin LF, et al. (2019) Optic Perineuritis Secondary to Sjogreen’s Syndrome: Case Report. Int J Brain Disord Treat 5:031.

Sun JY, Liu Z, Zhao P, Liu T. Optic neuritis as an initial presentation of primary Sjögren syndrome: A case report and literature review. Medicine (Baltimore). 2016;95(44):e5194. doi:10.1097/MD.0000000000005194

Tang WQ, Wei SH. Primary Sjögren’s syndrome-related optic neuritis. Int J Ophthalmol. 2013;6(6):888-891. Published 2013 Dec 18. doi:10.3980/j.issn.2222-3959.2013.06.26

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Dr. Arsia Hanif has been a meritorious Healthcare professional with a proven track record throughout her academic life securing first position in her MCAT examination and then, in 2017, she successfully completed her Bachelors of Medicine and Surgery from Dow University of Health Sciences. She has had the opportunity to apply her theoretical knowledge to the real-life scenarios, as a House Officer (HO) serving at Civil Hospital. Whilst working at the Civil Hospital, she discovered that nothing satisfies her more than helping other humans in need and since then has made a commitment to implement her expertise in the field of medicine to cure the sick and regain the state of health and well-being. Being a Doctor is exactly what you’d think it’s like. She is the colleague at work that everyone wants to know but nobody wants to be. If you want to get something done, you approach her – everyone knows that! She is currently studying with Medical Council of Canada and aspires to be a leading Neurologist someday. Alongside, she has taken up medical writing to exercise her skills of delivering comprehensible version of the otherwise difficult medical literature. Her breaks comprise either of swimming, volunteering services at a Medical Camp or spending time with family.


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