title: Primary Sjögren's syndrome related optic neuritis.
abstract: OBJECTIVE
  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.
  
  
  CONCLUSIONS
  Primary SS related optic neuritis is less common and easily misdiagnosed. The conventional therapies for optic neuritis could not control the recurrence.
authors.affiliation: Department of Ophthalmology, the First Affiliated Hospital of General Hospital of Chinese People's Liberation Army, No.51 Fucheng Road, Beijing 100048, China.
authors.forename: Wei-Qiang
authors.initials: WQ
authors.lastname: Tang
authors.affiliation: General Hospital of Chinese People's Liberation Army, Beijing 100853, China.
authors.forename: Shi-Hui
authors.initials: SH
authors.lastname: Wei
keywords: anti-aquaporin-4; optic neuritis; primary Sjögren's syndrome