Optic neuritis as an initial presentation of primary Sjögren syndrome

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.