Ramires Tiago Gama, Vieira Luísa, Riso Nuno, Moraes-Fontes Maria Francisca
Medicina 2, Hospital do Espirito Santo E.P.E, Évora, Portugal
Unidade de Doenças Auto-imunes/Medicina 7.2, HospitalCurry Cabral, Centro Hospitalar de Lisboa Central E.P.E, Lisboa, Portugal.
BMJ Case Rep. 2020 Jan 26;13(1):e229382. doi: 10.1136/bcr-2019-229382.
A 23-year-old woman with fever, oral ulcers, arthalgias and weight loss of 2-week duration suddenly developed blurred vision, with reduced visual acuity, cotton wool exudates and retinal vascular tortuosity. Laboratory testing revealed anaemia, lymphopaenia, positive antinuclear antibody and high anti-dsDNA antibody titre with low complement components. There was no evidence of infection, clinching the diagnosis of lupus retinopathy. Steroid therapy alone was highly effective and was also accompanied by a normalisation of haemoglobin and lymphocyte counts, after which azathioprine was added. Hydroxychloroquine was introduced after resolution of retinal changes. Immunosuppressive therapy was progressively tapered over the course of 12 months and then discontinued, and the patient remains in remission 48 months after the initial presentation. Our patient exemplifies a very rare manifestation of systemic lupus erythematosus. We emphasise the importance of its early detection and complexity of treatment in order to reduce visual morbidity.
一名23岁女性,发热、口腔溃疡、关节痛且体重减轻2周,突然出现视力模糊、视力下降、棉絮状渗出物和视网膜血管迂曲。实验室检查显示贫血、淋巴细胞减少、抗核抗体阳性、抗双链DNA抗体滴度高且补体成分低。没有感染证据,确诊为狼疮性视网膜病变。单独使用类固醇治疗非常有效,同时血红蛋白和淋巴细胞计数恢复正常,之后加用硫唑嘌呤。视网膜病变消退后加用羟氯喹。免疫抑制治疗在12个月内逐渐减量然后停用,患者在首次就诊后48个月仍处于缓解期。我们的患者体现了系统性红斑狼疮一种非常罕见的表现形式。我们强调早期发现的重要性以及治疗的复杂性,以降低视觉损害。