title: Optic Neuropathy Associated with Primary Sjögren's Syndrome: A Case Series.
abstract: To determine the diverse clinical features of optic neuropathy associated with primary Sjögren's syndrome in Korean patients.
  
  
  
  Five women with acute and/or chronic optic neuropathy who were diagnosed as primary Sjögren's syndrome were retrospectively evaluated. Primary Sjögren's syndrome was diagnosed by signs and symptoms of keratoconjunctivitis sicca, positive serum anti-Ro/SSA and/or anti-La/SSB antibodies, and/or minor salivary gland biopsy. All patients underwent a complete ophthalmologic examination.
  
  
  
  Among the five patients diagnosed as optic neuropathy related to primary Sjögren's syndrome, four patients had bilateral optic neuropathy and one patient was unilateral. The clinical course was chronic in three patients and one of them showed acute exacerbation and was finally diagnosed with neuromyelitis optica spectrum disorder. The other two patients presented as acute optic neuritis and one was diagnosed with neuromyelitis optica spectrum disorder. Sicca symptoms were present in four patients, but only two patients reported these symptoms before the onset of optic neuropathy. Patients showed minimal response to systemic corticosteroids or steroid dependence, requiring plasmapheresis in the acute phase and immunosuppressive agents for maintenance therapy.
  
  
  
  Optic neuropathy associated with primary Sjögren's syndrome may show variable clinical courses, including acute optic neuritis, insidious progression of chronic optic atrophy, or in the context of neuromyelitis optica spectrum disorders. Optic neuropathy may be the initial manifestation of primary Sjögren's syndrome without apparent sicca symptoms, which makes the diagnosis often difficult. The presence of specific antibodies including anti-Ro/SSA, anti-La/SSB, and anti-aquaporin-4 antibodies are supportive for the diagnosis and treatment in atypical cases of optic neuropathy.
authors.affiliation: *MD †MD, PhD Department of Ophthalmology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, Gyeonggi-do, Republic of Korea (all authors).
authors.forename: Eunoo
authors.initials: E
authors.lastname: Bak
authors.forename: Hee Kyung
authors.initials: HK
authors.lastname: Yang
authors.forename: Jeong-Min
authors.initials: JM
authors.lastname: Hwang
mesh_terms: D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000097:blood; D051401:Aquaporin 4 / Q000276:immunology; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008875:Middle Aged; D009901:Optic Nerve Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D020551:Pulse Therapy, Drug; D012189:Retrospective Studies; D012607:Scotoma / Q000175:diagnosis; D012859:Sjogren's Syndrome / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D014792:Visual Acuity / Q000502:physiology; D014794:Visual Fields / Q000502:physiology
chemical_list: C496030:AQP4 protein, human; D000974:Antibodies, Antinuclear; D051401:Aquaporin 4; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; C035356:SS-A antibodies; C035357:SS-B antibodies