Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2013 Dec 18;6(6):888-91.
doi: 10.3980/j.issn.2222-3959.2013.06.26. eCollection 2013.

Primary Sjögren's syndrome related optic neuritis

Wei-Qiang Tang  1 , Shi-Hui Wei  2
Affiliations

Primary Sjögren's syndrome related optic neuritis

Wei-Qiang Tang et al. Int J Ophthalmol. .

Abstract

Aim: To determine the clinical features, diagnosis and treatment of the primary Sjögren syndrome (SS) related optic neuritis.

Methods: The clinical data of 8 patients (12 eyes) with primary SS related optic neuritis were analyzed retrospectively.

Results: Eight of 128 consecutive patients with optic neuritis resulted from varied causes fulfilled the diagnostic criteria for the primary SS. They presented initially with the signs and symptoms of non-specific optic neuritis, and 5 patients presenting without dryness showed a chronic inflammation of submandibular gland or parotid gland, and lymphocyte infiltration was demonstrated by labial gland biopsy in 2 patients. There were serum positive titers for anti-Sjögren syndrome A (SSA) in 7 patients and anti-Sjögren syndrome B (SSB) in 8 patients. Anti-aquaporin-4 (AQP4) antibody was negative in all the 8 patients. Both glucocorticoids and immunosuppressive agent were administered, and visual acuity elevated in 8 eyes (66.7%), 3 patients (37.5%) recurred in the follow-up.

Conclusion: Primary SS related optic neuritis is less common and easily misdiagnosed. The conventional therapies for optic neuritis could not control the recurrence.

Keywords: anti-aquaporin-4; optic neuritis; primary Sjögren's syndrome.

PubMed Disclaimer

Figures

Figure 1
Figure 1. The optic disk was pale in the right eye of a patient with 3 times of relapse.
Figure 2
Figure 2. The optic disk was congested in a patient.
Figure 3
Figure 3. MRI findings at binocular optic neuritis. T1-weighted imaging showed the right optic nerve was much thickening.
See this image and copyright information in PMC

References

    1. Kim CS, Choi YD, Choi JS, Bae EH, Ma SK, Kim SW. EBV-positive diffuse large B-cell lymphoma in a patient with primary Sjögren's syndrome and membranous glomerulonephritis. BMC Nephrol. 2012;13:149. - PMC - PubMed
    1. Hu S, Vissink A, Arellano M, Roozendaal C, Zhou H, Kallenberg CG, Wong DT. Identification of autoantibody biomarkers for primary Sjögren's syndrome using protein microarrays. Proteomics. 2011;11(8):1499–1507. - PMC - PubMed
    1. Hamza N, Bos NA, Kallenberg CG. B-cell populations and sub-populations in Sjögren's syndrome. Presse Med. 2012;41(9 Pt 2):e475–483. - PubMed
    1. Seror R, Bootsma H, Bowman SJ, Dörner T, Gottenberg JE, Mariette X, Ramos-Casals M, Ravaud P, Theander E, Tzioufas A, Vitali C. Outcome measures for primary Sjögren's syndrome. J Autoimmun. 2012;39(1–2):97–102. - PubMed
    1. Theander E, Vasaitis L, Baecklund E, Nordmark G, Warfvinge G, Liedholm R, Brokstad K, Jonsson R, Jonsson MV. Lymphoid organisation in labial salivary gland biopsies is a possible predictor for the development of malignant lymphoma in primary Sjögren's syndrome. Ann Rheum Dis. 2011;70(8):1363–1368. - PMC - PubMed

LinkOut - more resources