Background: Primary Sjögren syndrome (pSS) is a progressive autoimmune disease that primarily affects exocrine glands. The clinical presentation of pSS may vary from an asymptomatic condition to severe skin symptom, resulting in a difficult and challenging diagnosis and treatment.
Methods and results: Here, we report a 47-year-old Chinese woman who lost vision in the right eye for 7 days. She had been misdiagnosed with primary optic neuritis for 3 months. After 3 months, the results of immunohistochemistry, salivary gland scintigraphy, and antibody tests proved the diagnosis of pSS. After an IV methylprednisolone treatment for 3 days (1.0 g/d), her final visual and perimetry outcome were satisfactory. A review of the relevant English literature based on PubMed encompassing dates up to July 2016 has been discussed.
Conclusion: Our finding and the literature review suggest that an early treatment may be beneficial but long-term disease may cause permanent irreparable damage.