- 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.
Examination:
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.
References:
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. doi.org/10.23937/2469-5866/1410031
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