Ghadiri Nima, Stanford Miles
Department of Ophthalmology, Medical Eye Unit, Guy's and St Thomas' Hospitals NHS Trust, London, UK
Department of Ophthalmology, Medical Eye Unit, Guy's and St Thomas' Hospitals NHS Trust, London, UK.
BMJ Case Rep. 2021 May 31;14(5):e240752. doi: 10.1136/bcr-2020-240752.
A 35-year-old woman presented with a constellation of systemic symptoms: rashes, weight loss, arthralgia and mouth ulcers. Six months afterwards, she experienced bilateral and sequential reduction in vision, and was found to have bilateral vaso-occlusive retinopathy, with critical macular ischaemia in the left eye. Her serological markers were consistent with a diagnosis of lupus. A lymph node biopsy confirmed Kikuchi-Fujimoto disease, a benign condition of unknown cause characterised by fever, cervical and axillary lymphadenopathy. Given that this overlap syndrome was associated with a number of systemic features and had affected the eyes, an immunosuppressive regime with rituximab was considered prudent. This rendered her vasculitis stable and non-progressive, and there were signs of partial retinal microvasculature recovery on optical coherence tomography angiography. There is increasing evidence of an overlap between Kikuchi-Fujimoto disease and systemic lupus erythematosus, which is associated with vaso-occlusive retinopathy. In these instances, a multidisciplinary approach is warranted, with consideration of appropriate treatment in order to prevent harmful sequelae of vasculitis. Our treatment with rituximab abated the disease process, although close follow-up is paramount to monitor results and side-effects of treatment.
一名35岁女性出现一系列全身症状：皮疹、体重减轻、关节痛和口腔溃疡。6个月后，她双眼视力先后下降，被发现患有双侧血管闭塞性视网膜病变，左眼黄斑严重缺血。她的血清学标志物与狼疮诊断相符。淋巴结活检确诊为菊池-藤本病，这是一种病因不明的良性疾病，以发热、颈部和腋窝淋巴结病为特征。鉴于这种重叠综合征与多种全身特征相关且累及眼部，考虑使用利妥昔单抗进行免疫抑制治疗是谨慎的。这使她的血管炎病情稳定且无进展，光学相干断层扫描血管造影显示视网膜微血管有部分恢复迹象。越来越多的证据表明菊池-藤本病与系统性红斑狼疮存在重叠，且与血管闭塞性视网膜病变有关。在这些情况下，需要采取多学科方法，考虑适当治疗以预防血管炎的有害后遗症。我们使用利妥昔单抗的治疗减轻了疾病进程，尽管密切随访对于监测治疗结果和副作用至关重要。
