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목적 : To determine the clinical features of optic neuropathy associated with primary Sjögren’s syndrome in Korean patients.
방법 : Five women with acute and/or chronic optic neuropathy who were diagnosed as primary Sjögren’s syndrome were evaluated retrospectively. Sjögren’s syndrome was diagnosed by signs and symptoms of keratoconjunctivitis sicca, positive serum anti-SSA(Ro) and/or anti-SSB(La) antibodies and minor salivary gland biopsy. All patients underwent a complete ophthalmologic examination.
결과 : Among the five patients, 3 patients had bilateral optic neuropathy and 2 patients were unilateral. The clinical course was chronic in 3 patients while one of them showed acute exacerbation. The other 2 patients presented as acute optic neuritis and one of them had neuromyelitis optica spectrum disorder. Among them, 4 patients had dry eye or dry mouth symptoms, but only 2 patients had reported these symptoms before the onset of optic neuropathy. All patients were treated by systemic corticosteroid and/or plasmapheresis, as well as immunosuppressive agents.
결론 : 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 syndrome, which makes the diagnosis often difficult. The presence of specific antibodies including anti-Ro/SSA, La/SSB and AQP4-IgG are supportive for the diagnosis and treatment in atypical cases of optic neuropathy.
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