Rapoport P, Merle H, Smadja D, Gerard M, Alliot E
Service d'Ophtalmologie, Centre Hospitalier Universitaire de Fort de France, Hôpital Pierre Zobda Quitman.
J Fr Ophtalmol. 1997;20(10):767-70.
A case of optic neuropathy and central nervous system disease developed in association with primary Sjögren's syndrome is described. Initial visual loss was partially reversible with intra veinous steroid therapy but four months later she underwent a controlateral visual loss and showed for the first time clinical manifestation of sicca syndrome. Fluorescein angiography showed signs of ischemic choroidopathy. Laboratory studies and histological findings on minor salivary gland biopsy was suggestive of primary Sjögren's syndrome. Attempts to taper oral steroid therapy resulted in worsening neurological disease and immunosuppressive therapy by azathioprine was successfully added to her regimen. Because of therapeutics and prognosis consequences, primary Sjögren's syndrome should be considered in differential diagnosis of optic neuropathy.
本文描述了一例与原发性干燥综合征相关的视神经病变和中枢神经系统疾病。最初的视力丧失经静脉注射类固醇治疗后部分可逆,但四个月后她对侧视力丧失,并首次出现干燥综合征的临床表现。荧光素血管造影显示缺血性脉络膜病变的迹象。实验室检查和小唾液腺活检的组织学结果提示原发性干燥综合征。尝试逐渐减少口服类固醇治疗导致神经疾病恶化,成功地在她的治疗方案中添加了硫唑嘌呤免疫抑制治疗。由于治疗和预后的影响,原发性干燥综合征应在视神经病变的鉴别诊断中予以考虑。